Rheumatology Flashcards

(61 cards)

1
Q

Things to clarify when taking joint history?

A

Which joint is affected
Previous episodes affecting the same or other joints
Speed of onset of symptoms
Precipitating factors e.g. trauma
Previous surgery to that joint
Systemic symptoms especially infective symptoms such as fevers
Other associated symptoms e.g. rash, gastrointestinal upset
Past medical history e.g. gout, coagulopathies
Medications e.g. anticoagulants

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2
Q

Presentation of an acute swollen joint?

A

Joint swelling
Erythema of the affected joint
Increased warmth of the affected joint
Pain and tenderness
Stiffness of the joint
Deformity may be present (e.g. in traumatic injury)
An effusion may be detectable on examination
Reduced range of motion may be present, with or without pain and crepitus
Small wounds or skin breakdown may provide a portal for infection
Other joints should also be examined to look for a polyarthritis or a source of referred pain
General examination may reveal associated features e.g. gouty tophi, other traumatic injuries

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3
Q

Differentials for acute swollen joint?

A

Septic arthritis
Gout
Pseudogout
Trauma
Haemarthrosis
Reactive arthritis
Neuropathic joint
Infections

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4
Q

What is adult onset stills disease?

A

Idiopathic systemic inflammatory condition presenting with high fevers, polyarthritis, arthralgia and rashes

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5
Q

Epidemiology of adult onset stills disease?

A

1 in 100, 000
Commonly presents between 16 and 35 years of age

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6
Q

Aetiology of adult onset stills disease?

A

Genetics and environmental
Preceding infection; EBV, CMV, mycoplasma
Elevated IL1, IL6, IL18, activation of neutrophils, macrophages and T helper cells

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7
Q

Criteria to diagnose adult onset stills disease?

A

Major;
Fever >39 degrees lasting > 7 days
Arthralgia > 2 weeks
Typical rash
Leukocytosis

Minor; Sore throat
Lymphadenopathy/ splenomegaly
Liver dysfunction
Rheumatoid factor, ANA negative

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8
Q

Presentation of adult onset stills disease?

A

Symptoms;
Intermittent high fevers
Often follows a quotidian pattern (daily recurrent fevers, usually late in the day)
Fatigue and myalgia may coincide with febrile episodes
Characteristic macular or maculopapular salmon-pink rash
Also intermittent and comes and goes with the fever
Usually on the limbs and trunk
May affect the palms, soles and face
Not itchy
Arthritis or arthralgia
Sore throat

Signs;
Lymphadenopathy which may be mildly tender
Hepatomegaly
Splenomegaly
Signs of arthritis
Swelling, tenderness and erythema
Knees, wrists and ankles are most commonly involved
Joint destruction may be seen
Non-suppurative pharyngitis

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9
Q

Differentials for adult onset stills disease?

A

Malignancy
Viral infection
Bacteraemia
Vasculitides
SLE
Familial mediterranean fever
Adverse drug reactions

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10
Q

Investigations to diagnose adult onset stills disease?

A

Urinalysis; protein, leukocytes
ECG; cardiac involvement

FBC, CRP, ESR, LFT, U+E, ferritin, coagulation screen, blood cultures, rheumatoid factor, ANA, viral serology, troponin

CXR, CT, PET, cMRI, X-ray

Bone marrow biopsy

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11
Q

Management of adult onset still disease?

A

NSAIDs
Low dose corticosteroids +/- methotrexate
High dose corticosteroids +/- DMARDs
Anti-IL1; anakinra, anti- IL6; tocilizumab
Anti- TNF

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12
Q

Complications of adult onset stills disease?

A

Macrophage activation syndrome
Pericarditis
Pulmonary manifestations; pleural effusion, pulmonary arterial hypertension, interstitial lung disease
DIC
AA amyloidosis

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13
Q

Prognosis of adult onset stills disease?

A

Patients display different patterns of disease:
Single episode followed by lifelong remission
Recurrent acute episodes with intermittent periods of remission
Chronic disease, often with joint destruction due to erosive polyarthritis
Generally prognosis is good, especially with prompt recognition and treatment
Macrophage activation syndrome is the most common cause of death with an up to 50% mortality rate

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14
Q

What is ankylosing spondylitis?

A

Seronegative inflammatory arthritis involving axial skeleton

Radiographic axial spondyloarthritis, sacroilitis and non radiographic changes which may be seen on MRI

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15
Q

Epidemiology of ankylosing spondylitis?

A

1 in 1000 caucasian
Peak onset between 20 and 30 years
More common in men
Strongly inheritable

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16
Q

Presentation of ankylosing spondylosis?

A

Lower back and buttock pain
Pain elsewhere in the spine may also occur
Stiffness that is worse in the morning and with rest, and improves with activity
Patients may wake in the second half of the night with pain
Pain and stiffness respond to NSAIDs
Peripheral enthesitis (pain, stiffness and/or swelling of the Achilles, quadriceps or patellar tendons, as well as plantar fasciitis)
Peripheral arthritis occurs in up to a third of patients, commonly affecting the ankles, knees and hips

Extra-articular involvement;
Anterior uveitis (eye pain, redness and blurred vision)
Inflammatory bowel disease (e.g. diarrhoea, abdominal pain, rectal bleeding)
Osteoporosis (increased risk of fragility fractures)
Aortitis which may lead to aortic regurgitation (shortness of breath, fatigue, chest pain)
Upper lobe pulmonary fibrosis (shortness of breath, exercise intolerance, dry cough)
IgA nephropathy (haematuria, fatigue)
Systemic symptoms of weight loss and fatigue

Signs;
Restricted movement in the lumbar spine
Schober’s test can be used to assess this as follows:
Mark two points on the back (one at the level of the L5 spinous process and one 10 cm above this)
On forward flexion, the distance between the two points should increase by 5cm or more
If the increase in distance is <5 cm, this indicates restricted forward flexion
Hyperkyphosis of the thoracic spine may develop as the disease progresses
Reduced C-spine movements and fixed flexion deformities may be seen
This can be measured by asking the patient to stand with their back to the wall
A distance of > 2 cm between their occiput and the wall is abnormal
“Question mark posture” refers to the combination of thoracic hyperkyphosis, loss of the lumbar lordosis and flexion deformities of the neck and hips (seen in advanced disease)
Reduced chest expansion may be seen
Affected joints may be tender and swollen
Affected tendons may be tender, stiff or swollen

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17
Q

Differentials for ankylosis spondylitis?

A

Mechanical back pain
Osteoarthritis
Psoriatic arthritis
Enteropathic arthropathy
Reactive arthritis
TB
Malignancy

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18
Q

Investigations to diagnose

A

FBC, ESR, CRP, HLA-B27

Pelvic Xray; sacroiliitis, bilateral, symmetrcal, erosion of the sacroiliac joint
Lumbar Xray; squaring of the vertebral
MRI
USS
DEXA scan

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19
Q

Management of ankylosing spondylitis?

A

Smoking cessation
Physiotherapy
Occupational therapy
Bone protection
Monitor for CVD risk

NSAIDs; paracetamol/ codeine if contra-indicated
Biological DMARD
Anti- TNF; infliximab/ adalimumab

Surgery to correct spinal defects

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20
Q

Complications of ankylosing spondylitis?

A

Spinal deformities including fusion of the axial skeleton in severe cases
Limited mobility
Deterioration in mental health due to chronic pain and limitations in function
Sleep difficulties due to pain and stiffness
Osteoporosis and spinal fractures
Increased cardiovascular risk
Heart failure
Aortic regurgitation and other valvular disorders
Restrictive lung disease
Apical pulmonary fibrosis
IgA nephropathy
Anterior uveitis
Side effects from treatment e.g. immunosuppression with DMARDs, peptic ulcers with NSAIDs

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21
Q

Prognosis of ankylosing spondylitis?

A

Progressive disease with irreversible damage

Good prognostic factors include:

Low functional impairment at diagnosis
Young age at disease onset
Short disease duration
High inflammatory markers at diagnosis

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22
Q

What is APS?

A

Antiphospholipid syndrome (APS) is an autoimmune condition characterised by the presence of antiphospholipid antibodies with clinical manifestations of venous or arterial thrombosis and adverse pregnancy outcomes.

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23
Q

Epidemiology of APS?

A

Prevalence of APS in the UK is 50 per 100,000 in women and 9.8 per 100,000 in men
Onset is typically between the ages of 20 and 50
Antiphospholipid antibodies are found in 1-5% of the healthy population, and in 30% of people with systemic lupus erythematosus
Approximately 1 in 6 people under the age of 50 with strokes, deep vein thrombosis, myocardial infarct or recurrent miscarriages have APS

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24
Q

Aetiology of APS?

A

Primary disease

Secondary to SLE, Rheumatoid arthritis, sjogren’s syndrome, systemic sclerosis, dermatomyositis, malignancy, infection

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25
Criteria to diagnose APS?
Sapporo criteria Vascular thrombosis At least 1 episode of venous/arterial/small vessel thrombosis Not including superficial thrombophlebitis This should be unprovoked or provoked by a minor risk factor only Pregnancy morbidity with any of: 3 miscarriages < 10 weeks 1 miscarriage _<_10 weeks Premature birth < 34 weeks Laboratory criteria: On at least 2 occasions at least 12 weeks apart, any of the following are positive: Anticardiolipin antibodies Anti-beta2-glycoprotein I antibodies Lupus anticoagulant
26
Presentation of APS?
Cerebral; stroke, central venous sinus thrombosis Rashes Lung; PE, PHTN Cardiac; MI, mitral regurgitation Thrombosis in peripheral arteries DVT Obstetric complications; recurrent miscarriage, still birth, PET, IUGR Retinal thrombosis Adrenal infarction Budd- chiari syndrome Mesenteric ischaemia Avascular necrosis Nephropathy
27
Differentials for APS?
Factor V leiden Protein C or S deficiency Antithrombin III deficiency Malignancy Polycythaemia Multiple sclerosis
28
Investigations to diagnose APS?
Urine dip; proteinuria Patients need to have at least one of the following antiphospholipid antibodies present on testing on two occasions at least 12 weeks apart: Lupus anticoagulant Anticardiolipin antibody Anti-beta2-glycoprotein I antibody FBC, Clotting, U+E, anti-dsDNA, anti-smith Doppler USS, CT/ MRI , TTE
29
Management of APS?
Conservative; Smoking cessation Regular exercise Avoid alcohol Manage co-morbidities Medical; Warfarin; target 2-3, if recurrent thrombosis then 3-4 Consider adding aspirin, hydroxychloroquine
30
Complications of APS?
Catastrophic APS with rapid onset widespread small vessel thrombosis Vascular dementia Chronic PE/ pulmonary hypertension Renal failure
31
Prognosis of APS?
Prognosis of APS varies widely Overall survival is good - approximately 90-94% over 10 years However, significant disability may result from complications such as stroke and pulmonary hypertension With treatment, pregnant women have an 80% chance of a successful birth Catastrophic APS occurs in only 1% of patients, however mortality is approximately 50%
32
Management of catastrophic APS?
Treatment dose heparin High dose steroids IVIG or plasma exchange
33
Presentation of back pain?
Dull or sharp pain in the area between the lower ribs and the buttocks, which can lead to: Restricted movement and difficulty mobilising Sleep disturbance Distress and low mood Associated symptoms include: Stiffness Muscle spasms Sciatica (leg pain which may be accompanied by weakness, numbness or paraesthesias)
34
Epidemiology of back pain?
The majority of people have had an episode of low back pain (over 80% of the population) Approximately 90% of these cases are non-specific Incidence peaks at 50-55 years, although prevalence and disability is greatest in people aged 80-85 Worldwide, it is the leading cause of disability
35
Risk factors for back pain?
Physical work especially involving heavy lifting, bending or twisting movements Sedentary lifestyle with lack of physical activity Obesity Smoking Depression and/or anxiety Stressful life events including physical or emotional trauma
36
Complications of back pain?
Disturbed sleep Impacts on daily functioning including chores, work and leisure Depression and anxiety Decreased mobility and increased falls risk especially in older people Acute low back pain may become chronic
37
What is bechets disease?
Multisystem inflammatory disease causing mouth and genital ulceration, skin lesions, uveitis and arthralgia
38
Epidemiology of bechets disease?
Most common in turkish people Men and women are equally likely to develop Behcet's disease, but men tend to be more severely affected HLA-B51 is the most strongly associated genetic risk factor Age of onset is usually in the 20s or 30s, although around 20% of cases present in childhood
39
Presentation of bechets disease?
Systemic symptoms; Fatigue Malaise Myalgia Low-grade fevers Headaches Arthralgia (an inflammatory arthritis may also occur) Mucocutaneous; Recurrent oral ulceration Mouth ulcers- heal within 7-21 days Genital ulcers Skin lesions Erythema nodosum Acne like papulopustular lesions Superficial thrombophlebitis Ocular; Uveitis; red, painful, photophobia, vision loss, retinal vasculitis Pulmonary; Pulmonary artery aneurysm Dyspnoea, chest pain, cough Rupture can lead to haemoptysis, syncope, cardiogenic shock CNS; Headaches Personality changes Cerebellar signs Dysarthria Sensory changes Memory loss Meningoencephalitis Cerebral vein thrombosis Presents with headache, decreased consciousness, visual loss and nausea and vomiting Signs include papilloedema, focal neurological deficits, seizures and cranial nerve palsies Gastro-intestinal; GI bleeding and perforation Cardiovascular; DVT Pericarditis Vasculitis Coronary artery aneurysm Cardiomyopathy
40
Differentials for bechets disease?
HSV Sarcoidosis Reactive arthritis Crohn's disease SLE
41
Investigations to diagnose bechets disease?
Wound swabs Urinalysis FBC, CRP, ESR, HLA-B51, RF, ANA, ANCA Syphilis serology U+E, LFT MRI head, MR venography, angiography, CT chest
42
Complications of bechets disease?
Visual loss GI bleeding GI perforation Acute ischaemia of limbs or organs due to vasculitis or thrombosis Acute coronary syndrome Aneurysm rupture (e.g. in the lungs or the brain) Seizures Cognitive impairment is common especially in neuro-Behcets Psychological distress including depression and anxiety
43
Prognosis of bechets disease?
The typical disease course is with flares of disease with intervening periods of remission The disease often becomes less active over time Mortality is generally low but is higher in men and patients with an early age of onset However death may occur due to neurological, GI or cardiovascular involvement Immunosuppressive treatments are also associated with significant risks e.g. of infection
44
What is pseudogout?
Articular and periarticular calcium pyrophosphate deposition (CPPD) causes a spectrum of disease, ranging from asymptomatic radiographic changes, to acute arthritis (also known as "pseudogout") as well as a chronic inflammatory arthritis.
45
Risk factors for pseudogout?
Previous joint injury or surgery Hyperparathyroidism Haemochromatosis Hypomagnesaemia Hypothyroidism Family history (ANK human gene mutations may be present)
46
Epidemiology of pseudogout?
All forms of CPPD disease are most common in older patients 50% of people will have radiographic changes of CPPD by the age of 80 Men and women are equally affected
47
Presentation of acute pseudogout?
Typically presents with an acute monoarthritis (rarely, an oligoarthritis) The knee, wrist, shoulder and elbow are the most commonly affected joints The affected joint is swollen, erythematous, warm and tender with an effusion on examination Systemic symptoms include fever and malaise
48
Presentation of chronic gout?
Usually a polyarticular arthritis affecting the small joints of the upper and lower limbs May present similarly to osteoarthritis with knee involvement, but inflammatory flares and severe articular damage can differentiate May resemble rheumatoid arthritis with wrists and metacarpophalangeal joint involvement Symptoms and signs are of chronic intermittent swelling and pain of affected joints
49
Differentials for pseudogout?
Gout Septic arthritis Osteoarthritis Rheumatoid arthritis
50
Investigations to diagnose pseudogout?
Microscopy of synovial fluid; Positively birefringent rhomboid-shaped calcium pyrophosphate crystals Culture of fluid X-ray; chondrocalcinosis Calcium, PTH, ferritin, TFT, Magnesium
51
Management of pseudogout?
NSAIDs, colchicine, steroids Joint aspiration
52
Prognosis of pseudogout?
Acute CPP arthritis usually resolves within days to short weeks, although some flares may last months Chronic CPP arthritis can cause significant joint damage with subsequent disability In some cases of refractory chronic disease, biologic agents have been trialled (e.g. anakinra)
53
What is enteropathic arthritis?
Enteropathic arthritis is an inflammatory arthritis associated with inflammatory bowel diseases (IBD) such as Crohn's disease or ulcerative colitis. Arthritis is the most common extraintestinal manifestation of IBD, affecting approximately 20-40% of patients.
54
Presentation of enteropathic arthritis?
Axial spondyloarthritis; Gradual onset of lower back pain and stiffness Symptoms are worse in the morning and improve with exercise Buttock pain is also common Examination findings include restricted spinal movements, sacroiliac joint tenderness and reduced chest expansion Type 1 peripheral arthritis; Oligoarticular arthritis of the large joints (i.e. fewer than 5 joints are affected) Usually asymmetrical; may affect only one joint (a monoarthritis) Knees and ankles are affected more commonly than the hips and shoulders Inflammation is often transient and migratory episodes are self-limiting in 90% of cases without permanent damage Strongly associated with flares of IBD and with extra-articular manifestations (e.g. uveitis and erythema nodosum) Often associated with enthesitis, tenosynovitis or dactylitis Type 2 peripheral arthritis; Polyarticular (i.e. 5 or more joints are involved) inflammation of primarily the small joints Metacarpophalangeal joints, knees and ankles are commonly involved Arthritis is usually symmetrical Joint inflammation is typically independent of IBD activity and is often chronic and migratory, causing damage to affected joints Other important symptoms and signs; Enthesitis is characterised by pain and inflammation at sites of tendon insertion (eg. Achilles tendinopathy, patellar tendinopathy, plantar fasciitis) Dactylitis - inflammation of a whole finger or toe Gastrointestinal symptoms including abdominal pain or cramping, diarrhoea, blood/mucus in faeces Systemic symptoms such as weight loss and fevers Dermatological manifestations such as pyoderma gangrenosum or erythema nodosum Aphthous ulcers in the mouth Anterior uveitis (pain and erythema of the eye with blurring of vision)
55
Differentials for enteropathic arthritis?
Whipples disease Reactive arthritis Coeliac disease
56
Investigations to diagnose enteropathic arthritis?
Faecal calprotectin Stool microscopy and culture Arthrocentesis for synovial fluid analysis FBC, CRP, ESR, anti-TTG, RF, p-ANCA X-ray of affected joint MRI spine and sacroiliac joint Colonoscopy +/- OGD
57
Management of enteropathic arthritis?
Local steroid injection DMARD; methotrexate, sulfasalazine NSAIDs, oral steroids Anti-TNF
58
What is eosinophilic granulomatosis ?
rare granulomatous small and medium-vessel vasculitis associated with asthma and tissue eosinophilia. AKA churg- strauss syndrome
59
Epidemiology of eosinophilic granulomatosis?
EGPA is rare, with a prevalence of approximately 10-14 cases per million people worldwide Men and women are equally affected Mean age at diagnosis is 50 years old Approximately 40% of patients are ANCA positive, usually anti-MPO (p-ANCA)
60
Presentation of eosinophilic granulomatosis?
Adult onset asthma Chronic sinusitis Fevers, malaise, myalgia, arthralgia, fatigue Skin lesions; palpable purpura, skin nodules and petechiae Peripheral nerve involvement GI involvement Glomerulonephritis Cardiac involvement; myocarditis, coronary arteritis, heart failure
61