Rheumatology Flashcards
Describe Systemic Lupus Erythematosus, it’s features, investigations, and management
Multisystemic autoimmune disease may be idiopathic or drug induced. 9 times more common in women, usually women of child-bearing age of afro-caribbean or asian background. Associated with HLA B8, DR2, DR3.
Features: facial rashes (malar, discoid), photosensitvity, oral ulcers, arthralgia, myalgia, fever, alopecia, non-infective endocarditis, raynauds.
Tests: ANA+ve, Anti dsDNA +ve (most sensitive), ESR raised, normal CRP, may also have anti-Ro, anti-La, anti-Sm (most specific), and Anti-RNP, Reduced C3 + C4
Management
- Acute flares: IV cyclophosmide + high-dose predisolone
- Lupus nephritis: Cyclophosphamide or mycophenolate
- Maintenance: hydroxychloroquine,azothioprine, methotrexate, mycophenolate
- Belimumab has a role in active auto-antibody-positive SLE with disease activity index (SELENA-SLEDAI) of 10 or more despite standard treatment
- Rashes: sunblock and topical steroids
What are the diagnostic criterium for SLE?
At least 4 of:
- Malar rash
- Discoid Rash
- Skin photosensitivity
- Oral ulcers (usually painless)
- Non-erosive arthritis (similar to RA)
- Serositis (e.g. pleuritis, pericarditis)
- Renal Disorder (Nephrotic syndrome)
- CNS disorder e.g. Seizures or Psychosis
- Haemtological Disorder (haemolytic anaemia, leukopenia, lymphopenia, thrombocytopenia)
- Immunological Disorder (anti dsDNA, Anti-sm, Antiphospholipid antibody)
- ANA +ve (+ve in more than 95%)
What are the causes of drug-induced lupus?
skin and lung signs prevail can be due to >50 drugs. e.g. isoniazid, hydralazine, procainamide, quinidine, chlorpromazine, minocycline, phenytoin. It is associated with antihistone antibodies in 100%
Describe antiphospholipid syndrome, its features, associations, and management.
An acquired disorder characterised by a predisposition to both venous and arterial thrombosis, recurrent foetal loss, and thrombocytopenia. It may be primary or secondar.
Features: Antiphospholipid antibodies causes CLOTs
-Coagulation defect
-Livedo reticularis
-Obstetric complications (recurrent miscarriage
-Thrombocytopenia
-Prolonged APTT
Associations:
- SLE (Most common)
- Other autoimmune disorders
- Lymphoproliferative disorders
- Phenothiazines
Management:
- Thrombotic risk managed with low dose aspirin
- VTE events warfarin with target INR 2-3 for 2-6months
- Recurrent venous thrombosis, lifelong warfarin if already on warfarin increase INR to 3-4
- Arterial thrombosis life long warfarin target INR 2-3
Describe Livedo reticularis
pink-blue mottling caused by capillary dilation and stasis in skin venules. May be a sign of SLE and Vasculitis.
Describe Henoch-Schonlein Purpura, its features, management, and prognosis.
A IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Berger’s disease). HSP is usually seen in children following an infection.
Features:
- Palpable purpuric rash (with localised oedema) over buttocks and extensor surfaces of arms and legs
- Abdominal pain
- Polyarthritis
- Features of IgA nephropathy may occur e.g. haematuria, renal failure
Management:
- Analgesia
- Supportive, inconsistent evidence for immunosuppressants.
Prognosis:
- usually excellent, self limiting condition especially in children without renal involvement
- around 1/3 patients have a relapse.
Describe Vasculitis
An inflammatory disorder of blood vessel walls causing destruction or stenosis. It can affect any organ and may be primary or secondary due to SLE, RA, Hepatitis B/C, HIV. Categorised depending on size of vessel
Large: Giant Cell Arteritis, Takayasu’s Artertits
Medium: Polyarterits Nodosa, Kawasaki Disease
Small:
pANCA +ve: Microscopic polyangitis, glomerulonephritis, Churg-Strauss syndrome
cANCA +ve: Wegener’s Granulomatosis (GPA)
ANCA -ve: Henoch-Schonlein purpura, Goodpasture’s Syndrome and cryoglobulinaemia
Describe Giant Cell Arteritis (GCA), its presentation, tests, and management
Large vessel Vasculitis, common in the elderly (consider takaysu’s if under 55yrs) associated with Polymylagia Rheumatica (PMR).
Presentation: headache, temporal artery and scalp tenderness, jaw claudication, amaurosis fugax or sudden blindness.
Test: increased ESR (typically over 47) + CRP (typically greater than 2.45mg/dL), increased platelets, increased ALP and decreased Hb.
Management:
- Immediate Prednisolone 80mg/d PO due to risk of irreversible blindness.
- Aspirin 75mg due to increased stroke risk
- Temporal biopsy within 7 days of steroids (beware skip lesions)
Describe Polyarteritis Nodosa (PAN)
Medium vessel necrotising Vasculitis that causes aneurysms and thrombosis. Twice as common in men may be associated with Hepatitis B.
Tests often show increased WCC, mild eosinophilia, anaemia, increased ESR + CRP. Renal biopsy can be diagnostic.
Treatment is similar to Microscopic Polyangitis with BP control and corticosteroids/cyclophosphamide.
Describe Microscopic Polyangitis, its features and management.
It is a p-ANCA +ve small (medium) vessel vasculitis. presents with rapidly progressive glomerulonephritis +/- Pulmonary haemorrhage.
Features:
- Renal impairment (Raised creatinine, haematuria, proteinuria)
- fever
- Other systemic symptoms e.g. Lethargy, myalgia, weight loss
- Rash (palpable purpura)
- Cough, dysponea, haemoptysis
- Mononeuritis multiplex
Management:
-Similar to PAN with BP control and corticosteroids/cyclophosphamide. Rituximab may also be used
Describe Polymyalgia Rheumatica (PMR), its features, investigations, and management
Linked with Giant Cell Arteritis but not a true Vacsculitis. Usually occurs in over 50
Features: bilateral aching, tenderness and morning stiffness of shoulders and proximal limb muscles. Not associated with weakness.
Investigations: raised CRP and normal or raised ESR. 30% have increased ALP. CK should be normal and can help distinguish from myopathy
Management:
- Predisolone 15mg/d PO (expect dramatic response within 1 week else seek other diagnosis (Differentials: RA, polymyositis, hypothyroidism, osteroarthritis, malignancy, spinal stenosis)
Describe Takayasu’s Arteritis
Large vessel vasculitis rare outside of Japan that affects the aorta and its branches. Often affects women age 20-40yrs. Aortic arch is often affected giving symptoms such as dizziness, visual changes, weak arm pulses.
Complications include aortic regurgitation, aortic dissection, ischaemic stroke, ischaemic heart disease. Also increased BP due to renal artery stenosis.
Describe Kawasaki Disease and its symptoms.
Medium vessel vasculitis of children similar to PAN. Median age is 10 months. Can lead to coronary artery aneurysm and infarction.
Symptoms: Child may present with bilateral non-purulent conjuncitivits, pharyngeal injection, strawberry tongue, pyrexia, diarrohea, neck lymphadenopathy, rash, palmar erythema, fingertip desquamation.
Describe Churg-Strauss Syndrome and its features.
Aka Allergic Granulomatosis with Polyangitis. pANCA +ve triad of adult-onset asthma, eosinophilia, and vasculitis affecting the lungs, nerves, heart and skin.
Features: Asthma, Eosinophilia over 10%, Paranasal sinusitis, mononeuritis multiplex, pANCA +ve in 60%, focal segmental glomeurlonephritis.
Leukotriene receptor antagonists may precipitate the disease.
Describe Wegner’s Granulomatosis (Granulomatosis with Polyangitis GPA), its symptoms, and management.
cANCA +ve necrotizing small vessel vasculitis. It mostly affects upper respiratory tract, lungs and kidneys.
Symptoms: Upper airways disease is common with nasal obstruction, ulcers, epistaxis, or destruction of nasal septum causing saddle nose deformity. Pulmonary involvement may cause cough, haemoptysis, pleuritis.
Management:
- steroids
- cyclophosphamide in steroid-resistant.
- consider plasma exchange in severe cases.
- Treatment response may be monitored by ANCA PR3 antigen
Describe Goodpasture’s Disease, its features, investigations, and management.
ANCA -ve small vessel vasculitis affecting kidneys and lungs, caused by anti-glomerular basement membrane.
Features: rapidly progressive glomerulonephritis (Nephritic syndrome +/- renal failure) + lung symptoms (haemoptysis)
Investigations: Renal biopsy shows crescentic glomerulonephritis that is rapidly progressive and there is linear basement membrane IgG deposition. Broncho-alveolar lavage May show haemosiderin laden macrophages.
Management:
- high dose IV steroids +/- cyclophosphamide
- Dialysis may be neccesary
What is cyroglobulinaemia, what conditions is it associated with?
Condition in which blood contains cyroglobulins (proteins that become insoluble at low temperatures).
They may be present in Mycoplasma Pneumonia, Post-streptococcal Glomerulonephritis, Multiple Myeloma, SLE, RA, Hepatitis B+C, HIV.
Describe Sjogrens syndrome its features, tests and treatment
It is a chronic inflammatory autoimmune disorder which may be primary (Onset 40-50yrs 9 times more common in women) or sencondary associated with connective tissue disease (RA, SLE, systemic sclerosis). There is lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands. Associated with increased risk of non-Hodgkins lymphoma
Features: decreased tear production, dry eyes, decreased salivation, parotid swelling, other glands are affected causing vaginal dryness. Systemically raynauds may present and lung, liver, joint and kidney involvement. It is associated with other autoimmune disease.
Tests: Anti-Ro (40%) and La +ve (26%), ANA +ve (75%), RF+ve (38%)
Managment: artificial saliva and tears, frequent drinks. NSAIDs and hydroxychloroquine for arthralgia, immunosupression for severe systemic involvement
Describe Rheumatoid Arthritis, its presentation, signs, extra-articular manifestations, investigations and management.
RA is a chronic systemic inflammatory disease, characterised by a symmetrical, peripheral polyarthritis. It is twice as common in women.
Presentation:
- Typically presentation is symmetrical, swollen, painful and stiff small joints of the hands and feet. It is associated with early morning stiffness.
- May present suddenly with widespread arthritis
- May present as a recurring mono/polyarthritis of various joints (palindromic RA)
- May present with persistent mono-arthritis
- May present systemically with initially few joint problems
Signs: Early signs include swollen MCP, PIP, wrist or MTP joints (often symmetrical). Later joint damage occurs and there may be ulnar deviation of the fingers and dorsal wrist subluxation. Boutonniere and swan-neck deformities of the fingers or z-deformity of the thumb.
Extra-articular manifestations: nodules on elbows and lungs, lymphadenopathy, vasculitis, fibrosing alveolitis, pleural/pericardial effusions, Raynaud’s, carpel tunnel, osteoporosis, amyloidoss, splenomegaly (Felty’s syndorme = RA + Splenomegaly + neutropenia)
Investigations: RF+ve (~70%), high titre associated with severe disease, X-ray of hands may show LOSE:
-Loss of joint space
-Osteopenia (juxta-articular)
-Soft-tissue swelling
-Erosions
Anti-cyclic citrullinated peptide antibodies (anti-CCP) are highly specific for RA.
FBC may show anaemia of chronic disease and increased platelets. ESR and CRP are also raised.
Management: Disease activity measured using DAS28 aiming to reduce score to less than 3.
- early used of DMARDS and biological agents improve long term outcomes. NSAIDs are good for symptom relief but have no effect on disease progression.
- Sulfasalazine and Hydroxychloroquine are usually 1st line combination therapy with replacement of sulfasalazine with methotrexate if disease not controlled.
- Biological agents may be used if failure to respond to two DMARDs and as DAS28 score >5.1
- TNF-alpha inhibitors are first line e.g. infliximab, etanercept, adalimumab usually given in combination with methotrexate though etanercept and adalimumab can be used in those intolerant of methotrexate.
- B-cell depletion e.g. rituximab is next used in combination with methotrexate when above therapy fails.
- IL-1 and IL-6 inhibition e.g. tocilizumab (IL-6) can be used in combination with methotrexate when above steps have failed.
- T-cell disruption agents such as abatacept may be used in patients with severe RA who have not responded TNF-alpha inhibitors or rituximab.
Describe scleroderma (aka systemic sclerosis) the types and management
Systemic sclerosis features scleroderma (skin fibrosis) and vascular disease, it can be split into two main types:
Limited cutaneous systemic sclerosis: formally known as CREST syndrome:
-Calcinosis of subcutaneous tissues
-Raynaud’s,
-oEsophageal and gut dysmotility
-Sclerodactyly (swollen tight digits)
-Telangiectasia
Skin involvement is ‘limited’ to the face, hands and feet. It is associated with anticentromere antibodies in 70-80%. Pulmonary hypertension is often present subclinically. Generally a milder disease with less skin involvement slow onset and progression.
Diffuse cutaneous systemic sclerosis usually has a more rapid onset and may involve skin of the whole body with skin changes presenting early and rapidly and is associated with early organ fibrosis i.e. lung, cardiac, GI and renal. Associated with antitopoisomerase-1 (Scl70) antibodies in 40% and anti-RNA polymerase antibodies in 20%. Requires annual ECG and spirometry.
Management: There is no cure. Immuosuppresive regimens e.g. IV cyclophosphamide are used for organ involvement or progressive skin disease. Monitor BP and renal function, and ACE-i help reduce the risk of renal problems. Leading cause of death is progressive pulmonary fibrosis and pulmonary hypertension
What is Froment’s test?
A test for ulnar nerve function, patient makes a fist and then grips a piece of paper between thumb and index finger. Examiner attempts to pull the paper away. If the patient can hold the paper ulnar nerve is intact if the patient flexes the thumb to compensate for inability to hold paper this is considered to be a failed test and indicative of ulnar nerve palsy.
What is Allen’s test?
This test should be performed before attempting an ABG. It is a test for abnormal circulation in the hand.
-The hand is elevated and the patient is asked to make a fist and squeeze tightly for 30 seconds.
-Pressure is applied to the ulnar and radial artery.
-Still elevated the hand is opened and should appear blanched
-Pressure is removed from the ulnar artery and colour should return within 7 seconds.
If it does not this is a positive and colour does not return to the hand appropriately then ulnar artery supply to the hand is insufficient and an ABG should not be attempted on the radial artery.
What is Finklesteins test?
It is a test used to identify DeQuervains tenosynovitis in people with wrist pain. The therapist grasps the thumb and ulnar deviates the hand sharply pain in the distal radial side of the forearm is +ve test. A modified version involves the patient making a fist grasped around the thumb and ulnar deviating but the former is more sensitive when performed correctly.
Describe osteoarthritis, its signs and symptoms, tests and mangement
It is the commonest joint condition, three times more common in women, typically presenting >50yrs.
Signs and symptoms:
- Localised disease (usually knee or hip) typically pain on movement and crepitius, worse at the end of the day with background pain at rest.
- Generalised disease, commonly affected joints are the DIP, thumb carpo-metacarpal joints and the knees. There may be joint tenderness, decreased range of movement and mild synovitis. Nodal swellings (bouchards nodes at PIP and Herbedens nodes at DIP)
Tests: Radiograph shows LOSS:
- Loss of joint space
- Osteophytes
- Subarticular sclerosis
- Subchondral cysts
Management: Exercise and weight loss if obese. Analgesia Regular paracetamol +/- topical NSAIDs. Capsicum cream may be tried. Steroidal injections in severe cases. In hip or knee joint replacement if substantial impact on quality of life.
Describe Septic Arthritis, its risk factors, investigations and treatment
Consider septic arthritis in any acutely inflamed joint, as it can destroy a joint in under 24h. The knee is affected in >50% of cases. Hip commonly affected in kids, Kochers criteria allows differentiaton from irritable hip (1 point for each, non weight bearing, ESR over 40, Fever over 38.5, WBC over 12, 3 or 4 indicates 95% probability of sepsis)
Risk factors: pre-exisiting joint disease (especially RA), diabetes, immunosuppresion, chronic renal failure, recent joint surgery, prosthetic joints, IV drug abuse, age >80yrs
Investigations: Urgent joint aspiration for synovial fluid microscopy and culture.
Treatment: If in doubt start broad spectrum IV antibiotics until sensitivities known.
Describe Gout its causes, investigations, treatment and prevention
Gout typically presents with an acute monoarthropathy, with severe joint, inflammation, >50% occur at the MTP joint of the big toe (podagra). May affect other joints and may present as polyarthropathy. It is caused by deposition of monosodium urate crystals in and near joints and is associated with raised plasma urate. In the long term there may be deposition of urate as tophi in tendons and pinna of ear.
Causes: Hereditary, alcohol excess, diuretics, leukaemia, cytotoxics.
Investigations: Polarised light microscopy of synovial fluid shows negatively birefringent needle-shaped urate crystals.
Treatment: High dose NSAIDs or colchicine is slower but better if NSAIDs are contraindicated.
Prevention: weight loss, decrease alcohol intake, low-dose aspirin increase serum urate. If plasma urate increased 3 weeks after attack allopurinol may be used If more than 2 attacks in a year (SE include rash, fever, decreased WCC).
- In renal impairment febuxostat May have a role in treatment.
- Uricosuric agent may help E.g. fenofibrate, lorsartsan
Describe Pseudogout its risk factors, tests and management
Similar to gout, it causes an acute monoarthropathy, typically of larger joints and it is due to calcium pyrophosphate crystals.
Risk factors: Hyperparathyroidism, Hypothyroidism, Haemochromatosis, acromegaly, low magnesium, low phosphate, Wilson’s disease.
Tests: polarised light microscopy of synovial fluid shows weakly birefrigent rhomboid-shaped crystals. It is associated with soft-tissue calcium deposition on radiographs.
Management: Aspiration, rest, intra-articular steroids. Methotrexate and hydroxychloroquine have a role in chronic psuedogout.
What are the main types of Spondyloarthritides and name some of the common features
Types: Ankylosing Spondylitis, Enteric arthropathy, Psoriatic arthritis, reactive arthritis
Features:
- seronegativity (RF -ve)
- HLA B27 association
- Axial arthritis, pathology in spine and sacroiliac joints
- asymmetrical large joint oligoarthritis or monoarthritis
- enthesitis, inflammation of the site of insertion of tendon or ligament in to bone e.g plantar fasciitis, Achilles tendonitis, costochondritis
- dactyliitis inflammation of an entire digit due to soft tissue oedema and joint inflammation
- extra-articulate manifestations e.g. Anterior uveitis, psoriatic my rashes, oral ulcers, IBD.
Describe Fibromyalgia, its features, and it’s management
Fibromyalgia and chronic fatigue syndrome are part of a diffuse group of overlapping syndromes, sharing similar demographic and clinical characteristics, in which chronic symptoms of fatigue and widespread pain feature prominently.
Features: Diagnosis depends on pain that is chronic I.e. More than 3 months, and widespread I.e. Involves both left and right sides, above and below the waist and the axial skeleton, in the absence of inflammation, and the presence of pain on palpating of at least ‘11/18 tender points’. Additional features include morning stiffness (80%), fatigue (80%), poor concentration, low mood and sleep disturbance.
Management:
- first rule out more sinister causes e.g. RA, PMR, vasculitis, hypothyroidism, myeloma.
- explain that is a relapsing remitting syndrome, reassure there is no serious underlying pathology.
- CBT and graded exercise programmes improve function capacity and develop coping strategies.
- Pharmcological intervention include low dose amitriptyline 10-20mg at night, and pregabaln (150-300mg/12hr PO).
Describe Restless legs syndrome, its features, associations, and management.
Features: compelling desire to move legs, worse at night, and relieved by movement, unpleasant leg sensations worse at rest. May be associated with Paraesthesias e.g. crawling Or throbbing sensations
Associations: iron deficiency, uraemia, pregnancy, DM, polyneuropathy, RA, COPD.
Management:
- exclude cramps, positional discomfort, and local leg pathology
- dopamine agonists are commonly used e.g. pramipexole, ropinirole
- anticonvulsants, opioids and benzodiazepines may have a role e.g. Carbmazepine, benzodiazepines, gabapentin
