Rheum Flashcards

Question

What are the important conditions to rule out in acute monoarthritis?

Answer 1

It is important to make the diagnosis of septic arthritis as the damage to a joint can happen very quickly and the patient be left with long-standing joint issues and osteoarthritis in the future. Prompt action with aspirating the joint and starting antibiotics is important to save the joint. Remember to ask if the patient has previously had a joint replacement as an infected prosthetic joint is an essential diagnosis to make as soon as possible. The orthopaedic surgeons will likely take the patient to theatre for a full wash out and the prosthetic joint may need to be replaced.

Answer 2

Reactive arthritis is a sterile inflammatory arthritis occurring within 4 weeks of an infection.

Answer 3

male sex (male to female ratio of 1.5:1) early adulthood, commonly presents between the age of 20 and 40 HLA-B27 positive preceding sexually transmitted or gastrointestinal infection, most commonly Chlamydia, Shigella, Yersinia or Salmonella infection occurs up to a month before arthritis and is sometimes asymptomatic. a triggering infection is identified in about 50% of cases

Answer 4

Arthritis – tends to be a peripheral arthritis beginning 1–4 weeks after infection - commonly an asymmetrical oligoarthritis, but may be polyarticular/monoarticular - most commonly affects the large joints of the lower limbs Axial arthritis, particularly of the sacroiliac joints and lumbosacral spine is another presentation - this occurs in up to 40% of patients and is associated with HLA-B27 Dactylitis can occur in some cases constitutional symptoms including fever, weight loss, lethargy – these can be severe and mimic sepsis (which needs to be excluded) enthesitis manifesting as Achilles tendonitis or plantar fasciitis painless mucosal ulcers dark maculopapular rash on palms and soles, known as keratoderma blenorrhagica in men, the glans penis may have an eroded appearance, known as circinate balanitis ocular inflammation including conjunctivitis and anterior uveitis urethritis and sterile dysuria – inflammation of bladder and prostate may cause cystitis and prostatitis aphthous ulcers in the mouth anterior uveitis

Answer 5

Arthritis – tends to be a peripheral arthritis beginning 1–4 weeks after infection - commonly an asymmetrical oligoarthritis, but may be polyarticular/monoarticular - most commonly affects the large joints of the lower limbs Axial arthritis, particularly of the sacroiliac joints and lumbosacral spine is another presentation - this occurs in up to 40% of patients and is associated with HLA-B27 Dactylitis can occur in some cases constitutional symptoms including fever, weight loss, lethargy – these can be severe and mimic sepsis (which needs to be excluded) enthesitis manifesting as Achilles tendonitis or plantar fasciitis painless mucosal ulcers dark maculopapular rash on palms and soles, known as keratoderma blenorrhagica in men, the glans penis may have an eroded appearance, known as circinate balanitis ocular inflammation including conjunctivitis and anterior uveitis urethritis and sterile dysuria – inflammation of bladder and prostate may cause cystitis and prostatitis aphthous ulcers in the mouth anterior uveitis

Answer 6

In most patients, reactive arthritis a self-limiting condition that resolves spontaneously Relapse occurs in around 30–50% of cases, this can be due to re-exposure to an infective agent Persistence of disease is associated with positivity to HLA-B27

Answer 7

In cases caused by genitourinary infections, the underlying infection needs to be treated Treatment usually involves NSAIDs in severe cases, oral or intra-articular corticosteroids may be needed In patients with persistent disease, management is similar to that of all inflammatory arthropathies, including conventional synthetic and rarely biological DMARDs

Answer 8

Felty syndrome is a rare variant of rheumatoid arthritis, seen in less than 1% of cases. It is defined as a triad of highly active rheumatoid arthritis (often with extrarticular disease), splenomegaly and neutropenia.

Answer 9

The typical patient has history of >10 years of active disease, and is linked to a strongly positive rheumatoid factor and HLA DR4.

Answer 10

Management is the same as severe rheumatoid arthritis, with DMARDs and corticosteroids in the first instance, and anti-TNF biologics if these fail. All patients should also be vaccinated against influenza and pneumococcus as the splenomegaly is associated with functional hyposplenism.

Answer 11

Gout is a form of arthritis caused by the deposition of monosodium urate crystals in and around the joints. Developed countries have a much higher prevalence than less developed countries.

Answer 12

Acute gout commonly presents with arthritis of the 1st MTP (metatarsophalangeal) joint. It is characterised by sudden, severe attacks of pain, swelling, redness and tenderness in the joints, often the joint at the base of the big toe.

Answer 13

Gout is caused by the precipitation of uric acid crystals within the joint leading to an acute inflammatory response.

Answer 14

Male gender Age over 50 years Family history of gout

Answer 15

Obesity Hypertension Chronic kidney disease Diabetes Metabolic syndrome Medications: thiazide diuretics, ACE inhibitors and aspirin

Answer 16

Gout has a number of triggers/ precipitating factors which can cause an acute flare, these are generally due to increased production of uric acid, either through digestion of protein or increased cell turnover and protein degradation. Seafood/ protein binges - eating lots of high protein foods raises levels of uric acid Chemotherapy - increases cell break down Trauma and surgery - increases cell break down

Answer 17

Excruciating, sudden, burning pain in the affected joint Swelling, redness, warmth, and stiffness in the affected joint Asymmetric joint distribution Mild fever Tachycardia as a transient sympathetic response to the pain of an acute attack

Answer 18

The most important differentials of a monoarthropathy are: Septic arthritis Crystal arthropathy - gout/ pseudogout Inflammatory arthritis - rheumatoid arthritis and seronegative arthritis Septic arthritis should be excluded in any patient presenting with an acute, hot swollen joint, especially if they are systemically unwell. Diagnosis is made by aspirating the joint and sending the fluid for microscopy, culture and sensitivity.

Answer 19

Initial tests that should be performed in a patient with suspected gout is arthrocentesis with synovial fluid analysis. Needle-shaped monosodium urate crystals with negative birefringence confirm gout and differentiate it from pseudogout, and the fluid should also be sent for gram stain and culture to rule out septic arthritis. Other investigations can be considered: The uric acid level should be obtained at least 2 weeks after the attack as it may be low or falsely normal during the attack. Additionally, gout can develop with serum uric acid levels with the normal range. X rays of affected joints: The radiological features of gout includes normal joint space, soft tissue swelling and periarticular erosions. Ultrasound of affected joints

Answer 20

Positively birefringent rhomboid shaped crystals made of calcium pyrophosphate

Answer 21

Needle-shaped monosodium urate crystals with negative birefringence

Answer 22

The radiological features of gout includes normal joint space, soft tissue swelling and periarticular erosions

Answer 23

Gout is managed in terms of the acute attack, and then the ongoing management. NSAIDs, colchicine, steroids and paracetamol may all be used acutely, according to the patient's co-morbidities. The NSAID, INDOMETHACIN is traditionally used first-line. For patients with a high risk of gastro-intestinal side effects, past medical history of chronic kidney disease or heart failure, colchicine may be used. Diarrhoea is a side effect of colchicine and if intolerable to patients, intra articular administration of steroids may be considered. Importantly, intra-articular administration of steroids should not be undertaken if septic arthritis is suspected.

Answer 24

Following management of acute gout, lifestyle changes are important to avoid another attack - Reduction of alcohol consumption - Reduction of purine-based foods- meat and seafood Medications should be reviewed as the following drugs may cause hyperuricaemia: - Thiazides and loop diuretics - Low dose salicylates - Chemotherapy Allopurinol should be commenced at a low dose at least 1-2 weeks after following an attack.

Answer 25

Criteria for commencing prophylactic allopurinol: More than 2/3 attacks per year Tophaceous gout X-ray changes showing chronic destructive joint disease Urate nephrolithiasis Patient experiencing severe and disabling polyarticular attacks If allopurinol fails or is contraindicated, febuxostat can be used instead. It is important to remember that urate lowering therapy should be only be started at least one week after an acute episode has resolved as it may worsen the acute episode. In addition starting or increasing urate lowering therapy can itself cause a gout flare. For this reason, the first 3-6 months of urate lowering therapy are often covered with an NSAID or colchicine until the effective dose is reached. If the patient experiences an acute flare while on urate lowering therapy, they should continue the drug.

Answer 26

Giant cell arteritis, also known as temporal arteritis, is where the arteries, particularly those at the side of the head (the temples), become inflamed.

Answer 27

Giant Cell Arteritis classically presents with some combination of the following features in an elderly person: Temporal headache (giant cell arteritis (GCA) can cause blindness and stroke, therefore it should be considered in any headache in an elderly person.) Jaw claudication (pain on chewing food) Amaurosis fugax (transient monocular blindness, often described as a dark curtain descending vertically) Thickened, tender temporal artery on examination. It may be pulseless Scalp tenderness The onset can be acute or insidious. In addition, GCA and polymyalgia rheumatica (PMR) often occur together, and so symmetrical proximal muscle weakness and an oligoarthritis may occur.

Answer 28

Permanent monocular blindness Stroke

Answer 29

First line tests to order in suspected giant cell arteritis are inflammatory markers (particularly ESR), FBC and LFTS. Patients often have a normochromic normocytic anaemia, and about ⅓ have mildly abnormal LFTs. The definitive investigation is temporal artery biopsy. If negative on the side with symptoms, the asymptomatic side may also be biopsied. 3-5cm of the artery should be biopsied due to skip lesions (i.e the lesions are segmental and tend to skip parts of the artery). Therefore, a negative biopsy does not rule out disease. If large-vessel involvement is suspected, this may be diagnosed by conventional angiography, magnetic resonance angiography, or CT scan. Often patients with large vessel involvement have normal temporal artery biopsy.

Answer 30

Giant cell arteritis should be treated immediately with high dose steroids (60mg OD prednisolone) to prevent blindness and stroke. Once symptoms resolve, prednisolone is gradually tapered, usually over a long period of 1-2 years, because relapses can occur when tapering too quickly. If weaning off of steroids is problematic and steroid sparing agents may be used to lower the dose such as azathioprine. Due to the prolonged high corticosteroid dose bisphosphonates and proton pump inhibitors may be warranted to prevent osteoporosis and gastric ulcers. Low dose aspirin is usually also given to further reduce the risk of stroke and blindness.

Answer 31

The sacroiliac joints are the joints most commonly affected in ankylosing spondylitis. This is why the presenting complaint is frequently of lower back pain

Answer 32

Raynaud's phenomenon is caused by vasospasm of small arteries and arterioles that decrease blood flow to the skin. Raynaud's phenomenon is fairly common and can be idiopathic (occur on its own) or secondary to other conditions like systemic sclerosis, SLE, RA, Sjogrens, thrombocytosis, polycythaemia rubra vera (PCV). It is more common in females and affects 3% of the population.

Answer 33

It is characterised by cold induced colour change of the fingertips white- blue- red and this colour change is well demarcated.

Answer 34

Diagnosis is clinical, and investigations are carried out when an underlying cause is suspected. Examination is necessary to check for tissue loss including digital ulceration and gangrene. The first line pharmacological treatment is dihydropyridine calcium channel blockers which can reduce both frequency and severity of attacks. Other options include ACE inhibitors and IV prostacyclin. In extreme cases nerve blocks or digital amputation might be necessary.

Answer 35

Subchondral sclerosis, subchondral cysts, Loss of joint space, osteophytes

Answer 36

Loss of joint space, soft tissue swelling, subluxation, peri-articular osteopenia

Answer 37

The main feature of polymyositis is bilateral, proximal (hip and shoulder girdle) muscle weakness, developing over weeks to months. Muscle pain (myalgia) and tenderness may also be present in approximately 1/3 of patients, but variable weakness is the more prominent feature. Despite this, muscle bulk and reflexes are preserved until very late. As the disease progresses, other muscles may also become involved, resulting in the following: Pharyngeal or oesophageal muscles leading to dysphonia and dysphagia. Respiratory muscles can lead to poor ventilation with type 2 respiratory failure. Occurs in 40-60 year olds and is more common in females

Answer 38

Dermatomyositis causes the above muscular features of polymyositis with additional skin rashes, including any of the following (in rough order of frequency): Heliotrope rash -which is a lilac discolouration of the eyelid skin in addition to periorbital oedema Gottron's papules which are scaly, erythematous papules over knuckles and extensor surfaces of knees and elbows Macular erythematous rash generally found on the head and neck, trunk or hands Periungual (nailfold) erythema Cutaneous vasculitis which can present as rashes and lead to ulcers Calcinosis

Answer 39

The following may occur in either poly or dermatomyositis: Systemic upset (fever / tiredness / malaise / weight loss) Joints (polyarthralgia) Pulmonary (interstitial lung disease) Cardiovascular (Raynaud's phenomenon and myocarditis)

Answer 40

5-15% of cases of poly/dermatomyositis are secondary to an underlying malignancy and should be looked for, especially in dermatomyositis as the association is much stronger than for polymyositis. The cancers that are most commonly associated with PM/DM include: Lung Oesophagus Breast Colon Ovary

Answer 41

The three most important initial tests to perform on all patients in whom you suspect a diagnosis of dermatomyositis or polymyositis are: Muscle-derived serum elevation. Creatinine kinase is the most sensitive indicator and the level usually parallels disease activity, particularly in polymyositis. EMG is performed next. It confirms the myopathic pattern of the disorder and excludes a primary neurogenic disorder. Typical neurogenic records include short duration and low amplitude polyphasic motor units on voluntary activation. Muscle biopsy is required next for definitive diagnosis. In polymyositis, muscle biopsy shows endomysial inflammatory infiltrates and in dermatomyositis, it shows perivascular, perimysial inflammatory infiltrate.

Answer 42

Blood tests that are elevated include lactate dehydrogenase, aldolase, ALT and AST. The following is a mnemonic to help remember the elevated serum enzymes in both dermatomyositis (DM) and polymyositis (PM): DM and PM turn your muscles into CLAAA (clay) Creatine kinase Lactate dehydrogenase Aldolase ALT AST

Answer 43

Dermatomyositis can manifest as a para-neoplastic syndrome associated with visceral carcinomas - particularly gastric, colorectal, genitourinary and lung cancers. Therefore, in both polymyositis and dermatomyositis it may be necessary to look for an underlying malignancy with further investigations. Polymyositis can also manifest as a para-neoplastic syndrome - particularly in association with lung cancer.

Answer 44

Corticosteroids are the mainstay of treatment. They are started at high doses initially, creatanine kinase is then monitored to guide the rate of tapering the dose. Many patients need an additional immunosuppressant such as methotrexate or azathioprine as a steroid-sparing agent. Hydroxychloroquine occasionally helps with skin disease in dermatomyositis. As well as pharmacological therapy, physiotherapy is essential to rehabilitate patients.

Answer 45

Osteoarthritis (OA) can be thought of as natural "wear and tear" on joints over time. Therefore the typical history is of a very insidious onset (progressing over months or years) of joint pain and stiffness in the elderly. Large, weight bearing joints are usually affected, such as the knee, hip and lumbar spine. It also often simultaneously occurs in the distal and proximal interphalangeal joints of the hands. Most common joint disease worldwide 1 in 5 over 45 have it Risk factors for osteoarthritis include obesity and repetitive trauma to joints from occupation / sporting activities.

Answer 46

Pain in OA is worse with movement and towards the end of the day, and morning stiffness is not prolonged (usually <20 minutes). In contrast, pain in inflammatory arthritis tends to improve with movement, and morning stiffness is prolonged (>30 minutes). This is especially helpful to differentiate lower back pain of osteoarthritis from inflammatory diseases such as ankylosing spondylitis. In terms of peripheral arthritis, the insidious onset of osteoarthritis will usually differentiate it from an inflammatory cause.

Answer 47

As the architecture of the joint is damaged, joints usually display a reduced range of movement and may have a fixed deformity. There may be slight swelling over the joint, but it shouldn't be particularly hot or red. Crepitus may be felt. In the hands, characteristic Heberden's and Bouchard's nodes may be seen, which are hard, bony swellings on the distal and proximal interphalangeal joints respectively.

Answer 48

The x-ray features of osteoarthritis can be remembered with the mnemonic LOSS: Loss of joint space Osteophytes Subchondral cysts Subarticular sclerosis

Answer 49

Conservative: Weight loss and aerobic exercise are effective at reducing pain and maintaining joint function. Weight bearing exercise should be avoided however as it can accelerate the progression of the disease. Physiotherapy and occupational therapy input help to improve a patient's gait, mobility and independence, and walking aids may be useful. Pharmacological Management of Osteoarthritis: Analgesia, following the principles of the pain ladder - Paracetamol alone may be suitable for mild disease, but NSAIDs are particularly effective for pain from osteoarthritis. For knee osteoarthritis: first-line therapy is topical NSAID treatment. Paracetamol is not routinely offered. In more severe cases, intra-articular steroid injections give effective, but temporary (weeks), pain relief, and carry with them local and systemic risks associated with steroid therapy. They are often used to cover a patient during an important life event or holiday. There is a limit to how many steroid injections a patient can have in a year. Surgical Management: Joint replacement (arthroplasty) may be required in severe cases (providing that they are fit enough for the procedure). It usually provides excellent pain relief, but will not improve the function of the joint. Other surgical procedures may be used in certain situations such as joint fusion or excision.

Answer 50

Systemic lupus erythematosus (SLE) is a multi-system auto-immune disease manifesting differently in different patients.

Answer 51

The classical presentation of SLE usually has some combination of: Systemic upset (fever/myalgia/fatigue/weight loss). Joint and/or skin involvement. Photosensitivity, which is the most common dermatological feature of SLE. This especially occurs in the form of a malar rash (symmetrical, butterfly shaped rash across both cheeks). Discoid rash (round, raised plaques). Mouth / nose / genital ulcers. Hair loss. Cutaneous vasculitis, which manifests as splinter haemorrhages / purpura. Presentations involving joints Arthritis (non-erosive) or arthralgia. The usual presentation is a polyarthritis which may be symmetrical or asymmetrical. Cardiovascular presentations Raynaud's phenomenon. Pericarditis, which is the most common cardiac feature of SLE. Myocarditis, which can present as arrhythmias and heart failure. Increased cardiovascular disease risk. Respiratory presentations Pleurisy and pleural effusions. Pneumonitis, which in the acute form can mimic pneumonia, chronic pneumonitis causes pulmonary fibrosis. Renal presentations Lupus nephritis, which is the most common cause of lupus-related death. It can be asymptomatic for a time before it presents as either nephritic or nephrotic syndrome. Patients should be monitored regularly for renal disease. This monitoring includes checking blood pressure (in case of sudden hypertension) and urinalysis (for proteinuria or haematuria). Neurological presentations Seizures. Migraines. Peripheral neuropathies. Psychiatric symptoms including psychosis or depression or anxiety. Haematological presentations Anaemia of chronic disease (common). Lymphopenia (common). Autoimmune haemolytic anaemia. Thrombocytopenia. Leucopoenia. Gastrointestinal presentations Peritonitis (aseptic). Hepatosplenomegaly.

Answer 52

It follows a relapsing-remitting course and organ involvement tends to become prominent in flares. Flares can be triggered by: Oestrogen-containing contraception. Overexposure to sunlight. Infections. Stress.

Answer 53

SLE patients can frequently develop the following conditions: Secondary Sjogren's syndrome Secondary anti-phospholipid syndrome Mixed connective tissue disease (features of SLE, systemic sclerosis and poly/dermatomyositis)

Answer 54

DIL presents as a milder form of SLE and is triggered by chronic use of certain drugs for over one month. Crucially symptoms should not be present before starting the drug, and should stop after discontinuing the drug. Although DIL can present with any feature of SLE, the most common features are: Systemic upset (fever / fatigue / myalgia). Arthritis (non-erosive) or arthralgia. Serositis in the form of pleurisy or pericarditis. Dermatological features of SLE such as the characteristic malar rash are less common in DIL, and SLE renal disease (the most common cause of death in SLE) and neurological involvement are rare. While SLE usually occurs in females, DIL occurs in males and females equally.

Answer 55

Drugs that are definitely known to cause drug induced lupus (DIL) include: Sulfadiazine Hydralazine Procainamide Isoniazid Methyldopa Quinidine Minocycline Chlorpromazine

Answer 56

Blood tests: DIL patients may be positive for ANA, but they should not be positive for the SLE specific autoantibodies Anti-dsDNA and Anti-sm. They may be positive for Anti-histone however, which is considered specific for DIL. Inflammatory markers may be raised.

Answer 57

FBC and clotting screen - a prolonged partial thromboplastin time (PTT) would suggest the presence of lupus anticoagulant and should prompt checking of anti-phospholipid antibodies. SLE patients often have anaemia of chronic disease, but can also have haemolytic anaemia. U+Es and urinalysis - to screen for renal involvement ESR and CRP - suggestive of active disease (in the absence of infection) Anti-nuclear antibody (ANA) - antibody to double stranded DNA (Anti-dsDNA) and Anti-Smith antigen. Complement (low C3 and C4) Positive ANA is not specific as it occurs in other connective tissue disease - Anti-dsDNA and anti-Smith antibodies are highly specific for SLE and often are confirmatory of the diagnosis. High titres of anti-dsDNA antibodies are markers of disease activity and correlate to worse outcome in lupus nephritis. ---------------------------------------------------- Other investigations CXR should be performed in all patients presenting with cardiopulmonary symptoms Pulmonary function tests show a restrictive pattern Pleural aspiration may be performed in patients to identify cause of pleural effusion and shows exudate Renal ultrasound may be carried out in patients with renal involvement to exclude other causes of renal impairment Renal biopsy is the most sensitive and specific test for lupus nephritis Plain X rays of affected joints show inflammation and non erosive arthritis in patients with joint manifestations Brain MRI may be done in patients with suspected cerebral lupus Echocardiogram can show pericarditis, pericardial effusion, pulmonary hypertension Serological findings Antinuclear antibodies (ANA) are sensitive but not specific Anti-dsDNA antibodies are specific and predict a poor prognosis with renal disease Other antibody findings Anti-Smith antibodies are antibodies directed against snRNPs, and are specific but not prognostic Anti-histone antibodies are sensitive for drug-induced lupus Anti-cardiolipin antibodies, which also trigger false-positive results on syphilis testing, and lead to a prolonged PTT Decreased C3, C4, and CH50 due to immune complex formation

Answer 58

The large number of potential symptoms can be difficult to remember, but the mnemonic A RASH POINts MD can help to remember the American College of Rheumatology Diagnostic Criteria and thus the important symptoms and investigations - presence of ≥4 supports the diagnosis: Arthritis / arthralgia. Renal disease. ANA positive. Serositis (pericarditis / pleurisy / pleural effusion). Haematological (haemolysis / low WCC / platelets / lymphocytes). Photosensitivity rash. Oral ulcers. Immunological tests positive (anti-dsDNA, anti-Sm, anti-phospholipid). Neuropsychiatric (seizures, psychosis). Malar rash. Discoid rash. Note: There is now a move towards using the SLICC Classification Criteria for SLE. Although similar to the above criteria it requires ≥4 findings with at least one clinical feature and one laboratory test (e.g. ANA, anti-DNA, anti-Sm, anti-phospholipid, low complement, direct Coomb's test) present.

Answer 59

**Preventative treatment** Avoiding triggers: - Oestrogen-containing contraception. - Overexposure to sunlight. - Infections. - Stress. **Pharmacological management** Management will be consultant lead and depends on the severity of the disease: NSAIDs and hydroxychloroquine are the mainstay of SLE treatment and may be sufficient for mild disease alone ± short courses of corticosteroids for flares. More prominent organ involvement may require long-term corticosteroids, usually in combination with a DMARD as a 'steroid sparing agent' to reduce the steroid dose. Severe flares causing serious renal, neurological or haematological effects may require high dose corticosteroids in combination with immunosuppressants. **Cyclophosphamide** is effective and is the usual choice. **Adjuvant therapy in SLE** Renal disease: Monitoring blood pressure and urinalysis (proteinuria or haematuria) to detect lupus nephritis early is essential. Hypertension should be treated aggressively, usually with ACE-inhibitors. Full renal failure will require corticosteroids and cyclophosphamide. Thrombocytopenia or leucopoenia: IV immunoglobulin infusions may be beneficial. Antiphospholipid syndrome: Antiplatelets or anticoagulation. Seizures: Anti-epileptic medication may be needed.

Answer 60

Many patients have chronic symptoms and the symptoms of an emergency (like Lupus Glomerulonephritis) may be subtle to spot. If Lupus Glomerulonephritis is untreated, patients will develop renal damage. This is the reason why all patients with SLE have a urine dipstick test to assess for proteinuria. If it is frankly nephrotic (proteinuria, hypoalbuminemia and oedema), an urgent admission is required. The mainstay of treatment is high dose steroids plus immunosuppression.

Answer 61

Cerebral Lupus can have many different presentations. Patients can present with fits, psychosis, severe unremitting headaches or impairment of consciousness. Patients will often have other signs of active SLE disease. It is often difficult to distinguish the mild cases of Cerebral Lupus from depression or infection. The ESR and anti ds-DNA antibody titres will be raised whereas the complement C3 and C4 levels will be low. The C-Reactive Protein (CRP) levels will often not be markedly raised. Sometimes, the blood markers of active SLE disease and the MRI scan can be normal. The management of Cerebral Lupus consists of high dose steroids and immunosuppression (Cyclophosphamide/Azathioprine). The response may be very slow but eventually these patients often do very well with no long term damage.

Answer 62

Takayasu's arteritis is a chronic granulomatous vasculitis affecting large arteries: primarily the aorta and its main branches. It is most common in Asian women between 10 and 40 years old. Vascular inflammation can cause stenosis, occlusion, and aneurysm formation.

Answer 63

Symptoms of Takayasu's arteritis include an absent upper extremity pulse, discrepancy in blood pressure between the arms (>10 mm Hg), night sweats, arthritis, myalgia, skin nodules, visual defects, and stroke. Vascular examination reveals abnormalities in the peripheral pulses, bruits over the central pulses, and there may be aortic regurgitation suggestive of aortic root dilation.

Answer 64

Diagnosis is made by vascular imaging. Management: Steroids are the mainstay of treatment. As with all patients on long term steroid treatment, bone and gastric protection must be considered.

Answer 65

Septic Arthritis is a joint infection caused by bacteria or a virus that spreads to the fluid surrounding the joint. It needs to be treated immediately.

Answer 66

The most common organism that causes septic arthritis is Staphylococcus Aureus. Other organisms include: Gonococcus: commonest in young sexually active individuals Streptococcus Gram negative bacilli

Answer 67

Joint disease such as rheumatoid arthritis Chronic kidney disease Immunosuppression Prosthetic joints

Answer 68

Acutely inflamed tender, swollen joint Reduced range of movement Systemically unwell Joint aspiration for Microscopy Culture and Sensitivity. The fluid itself will appear turbid and yellow, resembling pus. Blood tests show: high white cells, high ESR/CRP Blood cultures X-ray of the joint

Answer 69

IV antibiotics according to local guidelines Considering joint washout under general anaesthetic Physiotherapy after acute infection resolves

Answer 70

Osteomyelitis Arthritis Ankylosis fusion

Answer 71

The main side effects of orally taken bisphosphonates such as alendronic acid are oesophageal reactions including; oesophagitis, oesophageal ulcers, erosions and strictures which can present as odynophagia, dysphagia or new/worsening dyspepsia. Other rare, but memorable, side effects for all bisphosphonates include: Osteonecrosis of the jaw or auditory canal. Hypocalcaemia.

Answer 72

To reduce the risk of oesophageal reactions, the following specific advice should be given to patients: Tablets should be taken whole, with lots of water. They should be taken on an empty stomach at least 30 minutes before breakfast or any other oral medicines. After taking, patients should remain sat or stood upright for at least 30 minutes. Report any new upper GI symptoms (odynophagia, dysphagia, new/worsening dyspepsia).

Answer 73

A group of rare diseases that involve the hardening and tightening of the skin

Answer 74

Skin fibrosis is limited to the hands and forearms, feet and legs, and the head and neck. It is also known as CREST syndrome, because while any feature of diffuse cutaneous SSc can occur they primarily consist of the following: Calcinosis Raynaud's phenomenon oEsophageal dysmotility - dysphagia/GORD Sclerodactyly - bright shiny skin of hands and feet consistent with sclerodactyly. Fingers can be swollen and painful with reduced mobility and function. Patients are unable to put palm to palm in prayer sign. Telangiectasia - spider veins There is often also a family history of the disease. Anti-centromere antibodies are specific for limited cutaneous systemic sclerosis and present in 90% of cases.

Answer 75

Diffuse Systemic sclerosis is a multi-system autoimmune disease. Key features are abnormalities of blood vessels and fibrosis of the skin and internal organs. Skin involvement is over widespread areas at onset and is characterised by early visceral involvement. **Features in history and examination** Family history of systemic sclerosis Raynaud's phenomenon Digital pits or ulcers that can be painful and lead to functional disability Skin thickening, sclerodactyly and loss of function of the hands Telangiectasia Anti-Scl-70 antibodies are specific for diffuse systemic sclerosis

Answer 76

Dermatological features in systemic sclerosis Skin fibrosis presents as thickened plaques. In the initial stages they may appear inflamed (red/purple, puffy, itchy or painful) before settling into a more fibrosed state with the following features: Loss of normal skin creases. Hypo or hyper pigmented or shiny. Flexion contractures if overlying joints. Other skin features include: Sclerodactyly - thickening and contracture of an entire digit. Microstomia - restricted mouth opening, often with skin furrowing. Telangiectasia - small non-blanching red dots, often around the mouth. Calcinosis. In limited cutaneous SSc, these lesions are limited to the hands and forearms, feet and legs, and the head and neck, whereas they can occur anywhere in diffuse. In addition, most patients have some form of arthralgia. ---------------------------------------------------- Cardiovascular features in systemic sclerosis The vast majority of patients experience Raynaud's phenomenon. Pericarditis. Myocardial fibrosis which can cause heart failure and arrhythmias. ---------------------------------------------------- Gastrointestinal features in systemic sclerosis Oesophageal dysmotility. This can cause dysphagia or dyspepsia. Bowel hypomotility. This can cause bacterial overgrowth in the small bowel, or constipation in the large bowel. ------------------------------------------------ Respiratory features in systemic sclerosis Pulmonary fibrosis. Pulmonary hypertension. This causes a picture of right heart failure, with exertional dyspnoea and fatigue or weakness with signs of right heart failure (eg. raised venous pressure, peripheral oedema, cardiac heave). It can be asymptomatic for a long time before becoming rapidly apparent and so should be monitored for regularly with echocardiogram or diffusing capacity (DLco). Pulmonary hypertension is more common in limited cutaneous SSc, while pulmonary fibrosis and renal crises are more common in diffuse SSc. -------------------------------------------------- Renal features in systemic sclerosis Scleroderma renal crisis. This causes a rapidly progressive renal failure, usually with hypertension which can cause headaches and seizures. Unlike renal disease in systemic lupus erythematosus and rheumatoid arthritis, scleroderma renal crisis is not due to glomerulonephritis, and so nephritic or nephrotic syndromes do not usually occur. Blood pressure and renal function should be frequently monitored.

Answer 77

Inflammatory markers may be raised. Autoantibodies. Most patients are ANA positive, though this is not specific to SSc. Anti-Centromere antibodies are specific to limited cutaneous SSc. Anti-Scl-70 antibodies are specific to diffuse cutaneous SSc.

Answer 78

Renal monitoring should be done by monitoring blood pressure and renal function regularly. Pulmonary monitoring involved regular spirometry ± echocardiography. Spirometry Restrictive lung disease pattern seen in fibrosis. Transfer factor for carbon monoxide (TLco / DLco) which may be reduced in pulmonary hypertension. Echocardiography which may demonstrate pulmonary hypertension and right heart failure. If suspected, pulmonary hypertension can be formally diagnosed using right heart catheterisation.

Answer 79

Polymyalgia rheumatica is an inflammatory condition of unknown cause. It remains unclear whether it represents a form of giant cell arteritis or they are distinct conditions. It occurs exclusively in patients over 50. It is most common in patients with Northern European Ancestry.

Answer 80

Shoulder/ hip girdle stiffness (usually in the mornings) for >1 hour with associated inflammatory pain. Typically, these symptoms respond to treatment with corticosteroids within 72 hours and a therapeutic trial can be useful in diagnosis. Systemic symptoms including low grade fever, reduced appetite and weight loss, malaise

Answer 81

It is important to differentiate polymyalgia rheumatica from causes of a myositis, such as poly/dermatomyositis: Myositis causes bilateral proximal muscle weakness, while pain is either absent or mild. In polymyalgia rheumatica, pain and stiffness are prominent, but there should not be muscle weakness on examination (though this may be difficult to confirm as movements may be limited by pain.

Answer 82

The first line investigations for polymyalgia rheumatica are indicative of the differentials which include malignancy, endocrinopathy and metabolic bone disease. The most indicative investigations are ESR and CRP. The ESR can be markedly raised (>60mm/hr) The essential investigations are: FBC Urea and electrolytes LFTs Bone profile Protein electrophoresis Thyroid function tests Creatinine kinase Rheumatoid factor Urinalysis

Answer 83

The treatment for polymyalgia rheumatica is steroids. Unlike giant cell arteritis, steroids do not need to be started straight away giving time for an initial work-up to minimise risks of steroid use. After initial investigations, before commencing treatment for Polymyalgia rheumatica the following work up should be done: Prevention and treatment of steroid induced osteoporosis: DEXA scan those with high fracture risk. DEXA scanning is recommended in those without high fracture risk too. Screening for increased risk of adverse reaction to steroids before starting: diabetes mellitus, hypertension, history of peptic ulcer, osteoporosis, mental health history.

Answer 84

Colchicine - main adverse effect is diarrhoea

Answer 85

Disseminated gonococcal infection is a leading cause of acute septic arthritis in young sexually active patients and is known to be associated with tender necrotic pustules on the extremities. Most commonly the knee is involved, but involvement of smaller joints may occur, which is uncommonly simultaneous or migratory. It should be distinguished from reactive arthritis, which typically follows a symptomatic infection by 1-2 weeks and is generally less severe TENDER NECROTIC PUSTULES ON EXTREMITIES IS A SIGN OF STI

Answer 86

The vast majority of back pain in adults is mechanical. Mechanical back pain is in the lower back, typically occurs after straining, is relieved by rest, and may have paraspinal tenderness. mechanical back pain is a diagnosis of exclusion. The main rule when dealing with back pain is to exclude any of the following red flags which may indicate more sinister causes of back pain.

Answer 87

New onset age ≤20 or ≥55. Thoracic or cervical spine pain. Pain is progressive or not relieved by rest. Spinal (rather than paraspinal) tenderness.

Answer 88

Mechanical back pain should resolve within 6 weeks. If it doesn't, the patient should have an x-ray to exclude more sinister causes. If any red flags are present, some form of spinal imaging should be considered, (usually x-ray or urgent MRI if there is a neurological component), as well as any other tests for the most likely underlying cause as deemed appropriate eg. a myeloma screen if myeloma is suspected, followed by referral to orthopaedics or neurosurgery if necessary.

Answer 89

Features of ankylosing spondylitis back pain Prolonged morning stiffness. Improved with Movement. Alternating buttock pain. ---------------------------------------------------- Features of bone metastasis / myeloma back pain History of cancer. Systemic cancer symptoms eg. fever / weight loss / night sweats. ---------------------------------------------------- Features of discitis back pain Systemic infection symptoms eg. fever / weight loss / night sweats. Immunosuppression. IV drug usage. ---------------------------------------------------- Features of vertebral fracture back pain Violent trauma. ---------------------------------------------------- Features of cauda equina syndrome / spinal cord compression back pain New urinary or faecal retention or incontinence Poor anal tone or reduced perineal sensation (saddle anaesthesia) Bilateral lower limb neurology (alternating or simultaneous) ---------------------------------------------------- Features of nerve root compression back pain Normally unilateral (unless there is bilateral disease) Burning/shooting pain down the leg Positive sciatic stretch test Dermatomal stretch test positive Reduced reflexes

Answer 90

Cancers that commonly metastasise to the bone can be remembered with the mnemonic BLT with a Kosher Pickle: Breast Lung Thyroid Kidney Prostate.

Answer 91

COCP as risk of thromboembolitic event is increased

Answer 92

Rheumatoid arthritis is a commonon chronic inflammatory autoimmune disease. It is associated with significant morbidity which can be minimised if diagnosed and treated early. RA has a gradual onset over days to weeks and is considerably more common in females (3:1) and smokers. The peak onset is in 40-60 year olds.

Answer 93

Rheumatoid arthritis (RA) classically presents as: A symmetrical, polyarticular inflammatory arthritis involving the small joints of the hands (metacarpophalangeal and proximal interphalangeal joints), wrists, and feet. Other joints becoming involved later as the disease progresses. The distal interphalangeal joints (distal interphalangeal joints) are usually spared in RA however, which can help differentiate it from psoriatic arthritis which can present in a very similar fashion. The pain is inflammatory and as such is usually better with movement and associated with prolonged early morning stiffness (>30 min). Joints are swollen, red, warm, and tender on examination. Joint pattern involvement in rheumatoid arthritis is very variable however, and can take almost any form, including an acute monoarthritis and palindromic rheumatism, which causes recurrent, short lived (hours to days) episodes of arthritis in different joints before settling into a more permanent form.

Answer 94

Joint inflammation in RA can damage tendons, ligaments and the joint capsule, creating characteristic deformities on examination. Hand: Wrist subluxation. Metacarpophalangeal (MCP) subluxation. Swan-neck finger deformity (MCP flexion, PIP hyperextension, DIP hyperflexion). Boutonniere finger deformity (PIP flexion, DIP hyperextension). Ulnar deviation of proximal phalanges. Z-shaped thumb. Feet: Hallux valgus. Hammer toes. MTP subluxation. Occasionally extensor tendons of the hands and feet may rupture and require prompt surgical repair to maintain their function. RA spares the lumbar and thoracic spine but can involve the cervical spine, in particular the atlanto-axial joint - classically caysing neck pain radiating to the occiput and potentially altered sensation in the upper limbs Minor neck trauma in these patients can worsen a subluxation, and even cause upwards migration of the odontoid peg through the foramen magnum, resulting in spinal cord compression, compression of vertebral arteries, and even sudden death. C-spine imaging should be performed in any RA patient thought to be at risk. Usually a plain film will suffice and is often requested by anaesthetics pre-operatively as airway manoeuvres could worsen any existing disease. C-spine disease is usually treated surgically.

Answer 95

Carpal tunnel syndrome – causing pain, weakness and paraesthesia in the median nerve distribution of the hand. Tenosynovitis – typically of the flexor tendons in the hands, causing pain and swelling. Bursitis – typically of the olecranon (elbow) and sub-acromial (shoulder) bursae, causing pain and swelling.

Answer 96

Patients often feel generally unwell with RA, including low grade fevers, weight loss, and fatigue. ---------------------------------------------------- Haematological features Anaemia of chronic disease - this is common in RA (note that most DMARDs can also cause cytopenias as a side effect). Splenomegaly including Felty's syndrome (triad of RA, splenomegaly and neutropenia). Amyloidosis – most organs can be affected, classically the kidneys causing nephrotic syndrome. Generalised lymphadenopathy. ---------------------------------------------------- Dermatological features Rheumatoid nodules (firm, dark skin nodules, usually around sites of inflammation). Small vessel vasculitis causing nailbed infarcts and arterial leg ulcers. Raynaud's syndrome. ---------------------------------------------------- Ophthalmic features Keratoconjunctivitis sicca (dry eyes) – this can occur on its own, or as part of secondary Sjogren's syndrome with oral, genital and gastric ulcers. Episcleritis and scleritis. ---------------------------------------------------- Respiratory features Pleural effusions containing rheumatoid factor. Rheumatoid nodules may be seen on chest x-ray, and are asymptomatic. Pneumonitis leading to pulmonary fibrosis (note that this can also be a side effect of methotrexate). ---------------------------------------------------- Orthopaedic features Osteoporosis Cardiac features Pericardial effusions containing rheumatoid factor. Strong risk factor for cardiovascular disease. ---------------------------------------------------- Neurological features Peripheral neuropathy

Answer 97

Joint x-rays are often normal initially but later have the following typical features: Soft tissue swelling Periarticular osteoporosis. Juxta-articular erosions. Narrowing of joint space.

Answer 98

FBC – anaemia of chronic disease is common. Inflammatory markers – these are raised and can be used to monitor disease severity. Rheumatoid factor (RF) - considered specific for rheumatoid arthritis and can help support the diagnosis, though does not confirm it, and is present in 1% of the normal population. RA patients that are RF positive tend to have more systemic involvement and a worse prognosis. Anti-CCP – A positive anti-CCP is even more specific than RF for rheumatoid arthritis and can support the diagnosis, though does not confirm it.

Answer 99

Regular paracetamol and NSAIDs are the mainstay of reducing pain, swelling and stiffness. During flares, intra-articular or a course of oral steroids can be given. Physiotherapy - exercise prescription to help maintain muscle strength around the joint and thus range of movement Occupational therapy - provide advice and physical aids to avoid putting stress on joints during daily activities. Note - during acute flares, occupational therapists can also apply splints to joints. Surgery is usually reserved for severely damaged joints and may include joint arthroplasty, fusion or synovectomy

Answer 100

Disease modifying treatment slows the progression of the disease and joint destruction. They are indicated if the DAS28 (symptom severity) score is >5.1

Answer 101

DMARDs include a number of immunosuppressants, such as methotrexate, sulfasalazine, hydroxychloroquine and leflunomide. Current recommendations are to give DMARD monotherapy as first-line with the addition of a second DMARD if remission is not achieved. It is very important to commence DMARDs as early as possible after symptoms occur, ideally within 3 months. DMARDs can take several weeks to produce a response, and can cause mild immunosuppression as well as a number of important side effects which are unique to each drug.

Answer 102

For patients on disease modifying anti-rheumatic drugs, it is recommended that they receive the annual Influenza vaccine and the Pneumococcal vaccine every 5 years as per the schedule. Both vaccines are not live.

Answer 103

Hydroxychloroquine and/or Sulfasalazine with concomitant Folic Acid can be continued during pregnancy. These medications should be initiated and monitored by the Rheumatology team.

Answer 104

A biologic inhibits a specific target in the immune system thought to drive inflammation in RA. The most common are anti-TNF agents such as infliximab. Biologics are only used when the disease severity is severe (DAS28 > 5.1) despite long-term combination DMARD therapy. They are usually taken in addition to DMARDs. Biologics are expensive and have important side effects, most notably immunosuppression - they are usually discontinued if the patient does not see a significant improvement.

Answer 105

This is the treatment in suspected GCA with vision loss. High dose steroids are insufficient in this scenario

Answer 106

Trochanteric bursitis is the inflammation of the bursa overlying the greater trochanter of the femur. Causes include soft tissue trauma like a fall onto the hip as well as strain injury like hiking, differences in leg length, prolonged sitting and excessive running.

Answer 107

Symptoms include: Lateral hip pain (worse at night and aggravated by physical activity) Swelling A positive Trendelenberg (single leg stance) test

Answer 108

The management is mostly conservative and involves adequate analgesia. This may involve steroid injections. Physiotherapy is useful for stretching and strengthening exercises. Only as a last resort and in selected cases is bursectomy (removal of the inflamed bursa) considered.

Answer 109

Seronegative spondyloarthropathies are a heterogeneous group of inflammatory diseases.

Answer 110

The predominant features are: Axial involvement Peripheral joint involvement- predominantly asymmetrical and in lower extremities Enthesitis (inflammation at site where tendons and ligaments insert to bone - may manifest as Achilles tendonitis for example) High incidence of HLA-B27 but negative rheumatoid factor tests The unifying features of the spondyloarthropathies is inflammation involving entheses: enthesopathy. This is strongly associated with HLA-B27.

Answer 111

Ankylosing spondylitis Reactive arthritis Enteropathic arthritis Psoriatic arthritis Behcet's disease (resembles other spondyloarthropathies in certain respects but in other ways is quite distinct. It is associated with HLA-B51 rather than HLA-B27.)

Answer 112

Acute anterior uveitis (AAU) - 55% of cases are associated with HLA-B27. AAU is a common extra-articular manifestation of ankylosing spondylitis, and occurs more frequently in AS patients that are HLA-B27 positive. Juvenile idiopathic arthritis (JIA).

Answer 113

Male Aged 20-40 years old Inflammatory sacroiliac pain (stiffness and buttock pain worse in the morning) Enthesitis Dactylitis Urethritis or diarrhoea one-month preceding arthritis (for reactive arthritis) Anterior uveitis Psoriasis IBD

Answer 114

Granulomatosis with polyangiitis (Wegener's Granulomatosis) is a small and medium vessel systemic vasculitis

Answer 115

Patients will present with history and examination findings within the classic triad: - Upper respiratory tract involvement Chronic sinusitis Epistaxis Saddle- nose deformity - Lower respiratory tract involvement Cough Haemoptysis Pleuritis - Pauci-immune glomerulonephritis (both p- and c- ANCA positive) Haematuria Proteinuria with patients complaining of frothy urine

Answer 116

Another feature to look out for in initial investigation results are bilateral nodular and cavity infiltrates on chest X-ray.

Answer 117

Weak foot eversion Reduced ankle jerk

Answer 118

Hip flexion and adduction are affected in an L2 nerve root lesion

Answer 119

Knee extension and hip adduction are affected in an L3 nerve root lesion

Answer 120

Knee extension, foot inversion and foot dorsiflexion are affected in an L4 nerve root lesion

Answer 121

In an L5 nerve root lesion, foot inversion and dorsiflexion are affected

Answer 122

Staph. Epidermidis - Gram-positive facultative anaerobic bacterium and is part of the normal skin flora

Answer 123

Propanolol - treatment with Ca blocker or ramipril

Answer 124

Polyarteritis nodosa is a segmental transmural necrotizing vasculitis of medium-sized muscular arteries (does not involve arterioles, capillaries, or venules). Polyarteritis nodosa is most common in men and usually presents in patients aged 40-60. It can cause destruction of the arterial media and internal elastic lamina. It can also lead to aneurysm formation which can cause life-threatening haemorrhage. Associated with Hep B

Answer 125

Diagnosis of polyarteritis nodosa can be challenging because individual features are not distinguishable from those of many other diseases. The combination of constitutional symptoms and ischaemic symptoms in 1 or more organ system should raise the possibility of a systemic vasculitis. Diagnosis is based on the symptoms described by the patient, physical examination, various laboratory tests (to exclude other causes of the symptoms and point to PAN) and possibly biopsy of the affected area.

Answer 126

Kidney – renal failure Coronary – ischemic heart disease, acute myocardial infarction GI – abdominal pain, nausea, melaena Musculoskeletal – arthritis, myalgia, arthralgia CNS – eye and skin complaints (purpuric rash) The pulmonary vessels are not affected in PAN (Pulmonary vessels Are NOT affected). Note that polyarteritis nodosa has no association with ANCA antibodies.

Answer 127

Kidney – renal failure Coronary – ischemic heart disease, acute myocardial infarction GI – abdominal pain, nausea, melaena Musculoskeletal – arthritis, myalgia, arthralgia CNS – eye and skin complaints (purpuric rash) The pulmonary vessels are not affected in PAN (Pulmonary vessels Are NOT affected). Note that polyarteritis nodosa has no association with ANCA antibodies.

Answer 128

IgG4 related disease is recently described chronic multisystem fibroinflammatory disease characterised by a unifying histological feature: tumefacien lesions with dense lymphocytic infiltrate and storiform fibrosis.

Answer 129

Patients typically present sub-acutely with swelling of glandular tissue and allergic features. Commonly involved organs include the salivary and lacrimal glands, as well as the ear, nose and sinuses. Systemic upset is uncommon. Almost any organ may be affected. In the abdomen patients may develop autoimmune pancreatitis, as well as steroid-responsive sclerosing cholangitis (similar to primary sclerosing cholangitis) and retroperitoneal fibrosis. In the CNS, pituitary dysfunction and basal meningitis may occur. Vascular, thyroid, renal and pulmonary disease is also described.

Answer 130

Raised IgG4 is seen in 70% of cases (may be higher if serial dilutions performed). CRP is typically normal (the disease is not IL-6 driven), but the ESR elevated due to high levels of immunoglobulin. Complement levels are generally low and peripheral blood eosinophilia is consistent. Where symptomatic management is not sufficient, prednisolone is given in the short term. Rituximab has been considered for more long term management.

Answer 131

CFS (Chronic Fatigue Syndrome) is a chronic disabling disease of reduced productivity, and patients experience significant exhaustion and impairment following minimal physical or cognitive effort. Diagnosis is based on prolonged functional impairment, post-exertional malaise and appropriate exclusion of differentials. Viral and bacterial infections, including EBV have been investigated as initiating triggers to CFS, but the aetiology remains unclear.

Answer 132

Exertional exhaustion tends to be a good distinguishing feature vs fibromyalgia. Cognitive symptoms may also feature.

Answer 133

There is currently no curative treatment and treatments are mainly aimed at supportive management of symptoms.

Answer 134

Small joints of the hands or feet are affected. More than one joint is affected. There has been a delay of 3 months or longer between the onset of symptoms and the person seeking medical advice

Answer 135

Recurrent episodes of haemolysis basically mean high red blood cell turnover

Answer 136

anti-acetylcholine receptor antibody

Answer 137

Anterior ischaemic optic neuropathy is infarction of the posterior ciliary arteries which supply the optic nerve head is the most common mechanism of visual loss in giant cell arteritis. Classic findings on fundus examination include a swollen, chalky white, optic disc

Answer 138

This is the most suitable option when considering a patient's recent knee replacement surgery. When suspecting sepsis in a prosthetic joint, a referral should always be made to orthopaedics as joint aspiration should only be performed in a sterile operating theatre in these patients

Answer 139

Baseline as well as annual eye screenings are required for the rare complication of Hydroxychloroquine-induced retinopathy

Answer 140

In fibromyalgia, patients present with chronic widespread body pain and co-morbid symptoms including fatigue, mood and sleep difficulties. Widespread body pain means symptoms occur front and back, right and left and both sides of the diaphragm for at least 3 months, and tenderness to digital palpation at at least 11/18 tender points on examination. It is often co-morbid with rheumatological conditions. It is more common in females than males and most commonly presents in patients aged between 20 and 40 years old. There is often a family history of Fibromyalgia. There is unlikely to be a single cause for developing the condition, but current evidence indicates it is part of a CNS driven pain amplification syndrome.

Answer 141

Although there's currently no cure for fibromyalgia, there are treatments to help relieve some of the symptoms and make the condition easier to live with. Treatment tends to be a combination of: Medicine, such as antidepressants and painkillers Talking therapies, such as cognitive behavioural therapy (CBT) and counselling Lifestyle changes, such as exercise programmes and relaxation techniques

Answer 142

Familial Mediterranean fever is an autosomal recessive periodic fever syndrome caused by mutations in the Pyrin (MEFV) gene on chromosome 16. Fever, abdominal pain secondary to peritonitis, pleuritic pain due to pleuritis, arthritis, orchitis, erysipeloid rash

Answer 143

It presents in childhood or early adulthood (always under 25 years) and is characterised by recurrent bouts of fever, painful serositis and lower limb arthritis with an associated erysipelas-like rash. These typically last 1-3 days and are self limiting.

Answer 144

Symptomatic treatment for acute attacks is indicated, as well as long term colchicine therapy to reduce the risk of developing AA amyloidosis (a complication of chronic uncontrolled inflammation). In resistant cases, anti-IL-1 agents have been used and are beginning to show benefits in large international trials.

Answer 145

CT angiography is the first choice diagnostic test for Takayasu’s. It has high sensitivity and specificity (over 95%) for the diagnosis of Takayasu's arteritis. Imaging may reveal segmental narrowing of affected vessels and aortic aneurysms. It is preferable to catheter angiogram due to the reduced contrast load

Answer 146

Behcet's disease is a rare multi-organ disease caused by a systemic vasculitis. The cause is unknown. The disease is more common in people from Turkey, the Mediterranean and Japan. It affects around 1 in 300,000 people.

Answer 147

Recurrent oral ulceration Recurrent genital ulceration Uveitis Erythema nodosum

Answer 148

There is no specific test for Behcet's, and much of the work up is done to rule out differentials- and may include rheumatoid factor, antinuclear antibody (ANA), antinuclear cytoplasmic antibody (ANCA), as well as HLA B51. The Pathergy test can be used in certain scenarios but can be unreliable. This is characterised by exaggerated skin injury after minor trauma (skin prick using a sterile needle), when reassessed at 48 hours (see if a papule/ pustule forms) Behcet's is a multi system disease and CNS imaging may be appropriate for neurological symptoms, GI symptoms must be investigated with endoscopy to rule out IBD, and patients presenting with haemoptysis may require HRCT to rule out pulmonary aneurysm. Eye symptoms should be referred to an ophthalmologist.

Answer 149

Some patients only require treatment to cover a flare whereas other patients require immunosuppressive treatment with biologic drugs. Drugs used to treat Behcet's disease include steroids, colchicine, azathioprine, methotrexate and biologics.

Answer 150

SAK - Stomach, Asthma, Kidneys. ---------------------------------------------------- GI side effects Dyspepsia and upper GI ulceration. The risk is especially high in patients with a PMH of dyspepsia or GI ulceration, excessive alcohol intake or concomitant aspirin usage. If they are unavoidable in patients at a high risk, consider co-prescribing a PPI or using a COX-2 inhibitor instead. ---------------------------------------------------- Respiratory side effects A minority of asthma patients experience a deterioration with NSAIDs, so they should be used with caution in this group. ---------------------------------------------------- Renal side effects Nephrotoxicity - NSAIDs are most associated with pre-renal injury as they cause afferent arteriole vasoconstriction in the kidneys, though they can rarely cause acute interstitial nephritis. They should be withheld in AKI. ---------------------------------------------------- Other side effects Increased risk of cardiovascular events. Minor antiplatelet effect (should be avoided in active bleeding where possible).

Answer 151

COX-2 inhibitors, also known as coxibs, such as celocoxib are a newer alternative to traditional NSAIDs like ibuprofen or naproxen. Coxibs selectively inhibit COX-2 which confers a lower risk of GI ulceration and so may be preferred in patients at high risk of GI ulceration. However, shortly after they were first released, the coxib 'rofecoxib' was discontinued because it was found to increase the risk of cardiovascular events to a significantly higher extent than regular NSAIDs. This lead to a lack of uptake in coxib use, but more recent research has found that other coxibs, particularly moderate dose celocoxib, confers an equivalent cardiovascular risk to normal NSAIDs and are therefore safe to use in place of regular NSAIDs where necessary.

Answer 152

Corticosteroids are powerful immunosuppressants and have a high side effect profile Most important side effect is adrenal supression: If exogenous steroids are taken long-term the adrenal gland stops producing adequate levels of endogenous cortisol. If exogenous steroids are then stopped suddenly adrenal insufficiency can occur. In serious cases this can lead to shock, typically seen as hyperkalaemic hypotension. The management for this is to give more steroids usually as IV HYDROCORTISONE. Adrenal insufficiency can also occur during intercurrent acute illnesses as the body normally increases the production of cortisol in illness but cannot do this during steroid therapy as the adrenal axis is suppressed.

Answer 153

If they are taken for less than 3 weeks steroids can be safely stopped abruptly. If taken for longer than this, the steroids must be tapered down slowly. 'Sick day rules' - if acutely unwell double the usual dose of prednisolone or supplement IV hydrocortisone (usually 100mg 6-hourly) if unable to tolerate oral medication. If nil-by-mouth (or vomiting prednisolone before it can be absorbed), convert the increased daily dose of prednisolone to the equivalent IV hydrocortisone.

Answer 154

Reactivation of latent infections (TB / Hepatitis B/C / Herpes viruses): Have a low threshold for CXR to detect latent tuberculosis. If known to suffer with cold sores or shingles start high-dose acyclovir at the earliest sign. Raised risk of new infections: Any infection but particularly opportunistic ones such as candida. Infections are also generally worse but with potential masking of localising symptoms thus it is important to have low threshold for cultures. Aciclovir and varicella zoster immunoglobulin can be given if chickenpox exposure occurs. Live vaccinations are contra-indicated.

Answer 155

Dermatological / visual side effects of corticosteroids Cushingoid appearance with central obesity, buffalo hump, moon face. Acne. Thinned skin with striae. ---------------------------------------------------- Endocrine side effects Hyperglycaemia. Cushing's disease - Hypokalaemic hypertension with fluid and Na+ retention. Growth retardation in children. ---------------------------------------------------- Musculoskeletal side effects Muscle wasting. ---------------------------------------------------- Psychiatric side effects Mood swings. Worsening of underlying psychiatric condition. Can cause full steroid psychosis soon after administering. ---------------------------------------------------- Gastrointestinal side effects Dyspepsia. ---------------------------------------------------- Ophthalmic side effects Glaucoma. Cataracts. ---------------------------------------------------- Haematological side effects Raised white cell count.

Answer 156

The classic presenting symptom is haemoptysis following by deterioration in renal function. The age distribution is bi-modal, 20-30 years and 60-70 years

Answer 157

Goodpasture's syndrome Anti-glomerular basement membrane antibodies against type 4 collagen found within glomerular and alveolar basement membranes Granulomatosis with polyangiitis (Wegener's) Cytoplasmic anti-neutrophil cytoplasmic antibodies Churg-Strauss Peri-nuclear anti-neutrophil cytoplasmic antibodies SLE Antinuclear antibody, double-stranded DNA and anti-smith antibody are more specific Rheumatoid arthritis Rheumatoid factor, anti Anti-Cyclic Citrullinated Peptide (anti-CCP) is more specific

Answer 158

In enteropathic arthritis, joint involvement may present before, during or after a diagnosis of IBD. There are various patterns of joint disease.

Answer 159

Axial arthritis: Gradual onset of lower back pain and stiffness, worse in the morning, improves with exercise ---------------------------------------------------- Peripheral arthritis of IBD: Asymmetric, oligoarticular arthritis, predominantly of the lower limbs Often transient and migratory joint inflammation ---------------------------------------------------- Enthesopathy of IBD: Severe localised pain in specific areas of tendon insertion Achilles tendonopathy, patellar tendonopathy, plantar fasciitis

Answer 160

These may useful clues in the vignette Bowel symptoms to include abdominal pain, diarrhoea, weight loss, blood/ mucus in faeces Pyoderma gangrenosum and erythema nodosum Aphthous ulcers in the mouth Anterior uveitis Systemic symptoms including fever in a flare of disease

Answer 161

Inflammatory markers will likely be raised due to inflammation. Seronegative spondylarthropathies should all be rheumatoid factor negative. Seronegative spondylarthropathies are all associated with HLA-B27. A positive test can support the diagnosis, but not confirm or exclude it.

Answer 162

Affected joints do not generally show changes on X-ray. Pelvic X-rays may show sacroiliitis, and spinal X-rays may show squaring of vertebrae and syndesmophytes creating a "bamboo spine", as in ankylosing spondylitis

Answer 163

Corticosteroids (think CORTICOSTEROIDS): Cushing's syndrome Osteoporosis Retardation of growth Thin skin, easy bruising Immunosuppression Cataracts and glaucoma Oedema Suppression of HPA axis Teratogenic Emotional disturbance (including psychosis Rise in BP Obesity (truncal) Increased hair growth (hirsutism) Diabetes mellitus Striae

Answer 164

Indigestion Peptic ulcer disease, Increased risk of venous thrombo-embolus Peripheral oedema Slight increased risk of stroke and heart attack

Answer 165

Gastro-intestinal disturbance Folate deficiency - anaemia Immunosuppression Pulmonary fibrosis Liver toxicity Interstitial pneumonitis Rash Teratogenecity- Methotrexate is contraindicated during conception and pregnancy. The recommendation is a wash out of a few months (at least 3 months) before conception. In the event of a disease flare, low dose steroids are thought to be relatively safe. Note that high doses are associated with a small increased risk of the child having a cleft palate.

Answer 166

Myelosuppression Renal toxicity (Nephrotic syndrome) Mouth ulcers Photosensitivity Chrysiasis (skin discolouration)

Answer 167

Immunosuppression Reactivation of TB Allergic reaction, reaction at infusion site

Answer 168

Retinopathy Rash

Answer 169

Myelosuppression Nausea Rash Oral ulcers Decreased sperm count

Answer 170

Pseudogout (or "false gout") is a form of arthritis that results from deposits of calcium pyrophosphate crystals.

Answer 171

Advanced age Injury or previous joint surgery Hyperparathyroidism Haemochromatosis Hypomagnesaemia Hypophosphataemia

Answer 172

Inflammation and pain in one joint (usually monoarticular arthritis but may be oligoarticular)- commonly the wrist or shoulder that worsens over weeks Affected joints are acutely inflamed with swelling, effusion, warmth and tenderness Microscopy findings are Positively birefringent rhomboid shaped crystals made of calcium pyrophosphate

Answer 173

Acute episodes of pseudogout are treated very similarly to those of gout. Treatment is usually with a course of NSAIDs. If NSAIDs are contraindicated, a course of colchicine may be used instead. Colchicine is problematic because it is very prone to causing significant GI disturbances, especially diarrhoea. If both NSAIDs and colchicine are contraindicated, a short course of oral steroids, or an intra-articular steroid injection may be used. Unlike gout, there is no effective prophylactic treatment for pseudogou

Answer 174

Buerger's disease is a non-atherosclerotic vasculitis caused by occlusion in small and medium-sized arteries. It is most common in young male smokers of Mediterranean and Middle Eastern origin. It typically presents as an acutely ischaemic limb, without a background of peripheral claudication. Can be a cause of secondary raynaud's Investigations are aimed at excluding differentials (such as atherosclerotic disease and autoimmune disease). Arterial Doppler will confirm the absence of peripheral pulses in the affected limb. Further imaging (such as with arterial duplex or CT/MR angiography) will show non-atherosclerotic occlusion. Martorell's sign on arterial duplex describes the 'corkscrew'-shaped collateral vessels characteristic of Buerger's disease. Management is with smoking cessation ± vasoactive medication (such as nifedipine). If there is critical ischaemia the patient requires hospital admission, vasoactive medication and debridement of gangrenous tissue.

Answer 175

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) is a rare granulomatous small and medium vessel vasculitis.

Answer 176

Presentation can depend on the systems involved. In any patient with asthma, nasal polyposis and constitutional symptoms, Churg Strauss Syndrome should be a differential. The American College of Rheumatology has identified 6 criteria for the diagnosis of Churg- Strauss syndrome: Asthma Eosinophilia of >10% in peripheral blood Paranasal sinusitis Pulmonary infiltrates Histological confirmation of vasculitis with extravascular eosinophils Mononeuritis multiplex or polyneuropathy

Answer 177

BCG, MMR, Oral Polio & Typhoid, Yellow Fever

Answer 178

Pernicious anaemia Intrinsic factor antibodies are very specific Coeliac disease Anti-tissue transglutaminase antibodies (tta), IgA anti-endomysial antibody (IgA), anti-deamidated gliadin peptides (IgG) Antimitochondrial antibodies (IgM) Present in 95% of primary biliary cirrhosis patients Anti-smooth muscle antibodies (IgG) High titres are found in 95% of patients with autoimmune hepatitis

Answer 179

The main side effects of orally taken bisphosphonates such as alendronic acid are oesophageal reactions including; oesophagitis, oesophageal ulcers, erosions and strictures which can present as odynophagia, dysphagia or new/worsening dyspepsia. Other rare, but memorable, side effects for all bisphosphonates include: Osteonecrosis of the jaw or auditory canal. Hypocalcaemia.

Answer 180

To reduce the risk of oesophageal reactions, the following specific advice should be given to patients: Tablets should be taken whole, with lots of water. They should be taken on an empty stomach at least 30 minutes before breakfast or any other oral medicines. After taking, patients should remain sat or stood upright for at least 30 minutes. Report any new upper GI symptoms (odynophagia, dysphagia, new/worsening dyspepsia).

Answer 181

The cytoplasmic c-ANCA pattern, associated with antibodies to proteinase 3 (PR3) in >90% cases, more often seen in Granulomatous polyangitis (Wegener's granulomatosis). Cocaine users may also present with perforation of oro-nasal structures including the hard palate and nasal septum, and may also be c- ANCA and anti PR3 positive, but can be distinguished from GPA by positive human neutrophil elastase antibody testing. The perinuclear p-ANCA pattern, associated with antibodies to myeloperoxidase (MPO) in 80-90% cases, more often seen in Microscopic polyangitis and Churg Strauss syndrome

Answer 182

Dual positivity (to both p- and c-ANCA) should raise suspicion of a false positive result. Causes of dual positive results include: subacute bacterial endocarditis (and other chronic infections), haematological malignancy, inflammatory bowel disease, and drug induced vasculitis (including cocaine induced vasculitis).

Answer 183

I Hate Multiple Meds Causing Queer Problems; Isoniazid, Hydralazine, Methyldopa, Minocycline, Chlorpromazine, Quinidine, Procainamide Positive anti-histone antibody likely to be present in drug induced lupus

Answer 184

All patients with SLE needs to have a urine dipstick test to look for proteinuria (evidence of Lupus Glomerulonephritis)

Answer 185

Flucloxacillin 2g intravenous QDS

Answer 186

Anti-tuberculous treatment with pyrazinamide and ethambutol is known to reduce renal urate excretion and precipitate particularly troublesome attacks of gout

Answer 187

Allopurinol can acutely raise the level of urate (and precipitate or worsen a flare of gout) before lowering the levels of gout. Therefore covering with an NSAID is important for reducing the risk of another flair while allopurinol therapy is initiated

Answer 188

Dexamethasone 16mg (BD - as soon as possible to reduce oedema and cord compression), urgent neurosurgical referral, MRI whole spine

Answer 189

Hypermobility is the term used to describe the ability to move joints beyond the normal range of movement. Hypermobility syndrome is where, for a small percentage of the population, instead of being advantageous, hypermobility is associated with joint and ligament injuries, pain, fatigue and other symptoms.

Answer 190

Joint hypermobility by itself can be benign. In some sports, it can be beneficial (e.g. ballet, ice-skating, etc). When this causes pain, this becomes joint hypermobility syndrome. Patients can present with pain or stiffness in the joints or muscles, frequent sprains, dislocations of the joints, poor balance, thin stretchy skin and GI issues like diarrhoea or constipation.

Answer 191

The Beighton score is used to assess the joints and a score of 4 points makes hypermobility likely. It requires the performance of 5 manoeuvres, four passive bilateral and one active unilateral movements. A score of 4 points and pain in 4 or more joints for at least 3 months makes joint hypermobility syndrome likely.

Answer 192

There is no cure. The main treatment is strengthening the muscles and improving fitness to protect the joints. Physiotherapists and occupational therapists can give specialist advice. Paracetamol and ibuprofen can be prescribed as analgesia. Gentle low-impact exercise can help. Conversely, high impact or no exercise can make the symptoms worse. Straining a joint (gripping things too tightly, overextending joints) can also worsen symptoms.

Answer 193

Naproxen and other NSAIDS

Rheum Flashcards

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