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Year 4 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What disease is this autoantibody found and what does it test for?

RF

A
  • Disease:
    • RA 80%
    • SS 50%
    • SLE 20%
  • tests for
    • Autoantibodies (IgM>IgG>IgA) directed against Fc domain of IgG
    • Present in most seropositive diseases
    • Levels correlate with disease severity in RA
    • Non-specific
    • Present in IE, TB, hepatitis C, silicosis, sarcoidosis
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2
Q

What disease is this autoantibody found and what does it test for?

Anti-CCP

A
  • Disease:
    • RA 80%
  • Tests for:
    • In RA: anti-CCP more specific than RF
    • May be useful in early disease and to predict aggressive disease
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3
Q

What disease is this autoantibody found and what does it test for?

ANA

A
  • Disease
    • SLE 98%
    • Mixed connective tissue disease (MCTD) 95%
    • Sjögren’s syndrome 70-90%
    • CREST 80%
  • Tests for
    • Ab against nuclear components (DNA, RNA, histones, centromere)
    • Sensitive but not specific for SLE
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4
Q

What disease is this autoantibody found and what does it test for?

Anti-dsDNA

A
  • Disease
    • SLE 50-70%
  • Tests for:
    • Specific for SLE
    • Levels correlate with disease activity
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5
Q

What disease is this autoantibody found and what does it test for?

Anti-Sm

A
  • Disease
    • SLE <30%
  • Test for
    • Specific but not sensitive for SLE
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6
Q

What disease is this autoantibody found and what does it test for?

Anti-Ro (SSA)

A
  • Disease
    • Sjögrens syndrome 40-95%
  • Test for
    • Subacute cutaneous SLE and mothers of babies with neonatal SLE 25%
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7
Q

What disease is this autoantibody found and what does it test for?

Anti-La (SSB)

A
  • Disease
    • Sjögrens syndrome 40%
    • SLE 10%
  • test for:
    • Usually occurs with anti-Ro
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8
Q

What disease is this autoantibody found and what does it test for?

Antiphospholipid Ab (LAC, ACLA)

A
  • Disease
    • Antiphospholipid antibody syndrome (APLA) 100%
    • SLE 31-40%
  • tests for
    • By definition present in APLA
    • Only small subset of SLE patients develop clinical syndrome of APLA
    • If positive, will often get a false positive VDRL test
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9
Q

What disease is this autoantibody found?

Anti-Histone

A
  • Drug-induced SLE >90%
  • Idiopathic SLE >50%
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10
Q

What disease is this autoantibody found?

Anti-RNP

A

mixed connective tissue disease

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11
Q

What disease is this autoantibody found and what does it test for?

Anti-centromere

A
  • Disease:
    • CREST >80%
  • tests for:
    • Specific for CREST variant of systemic sclerosis
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12
Q

What disease is this autoantibody found?

Anti-topoisomerase I

A

Diffuse systemic sclerosis 26-76%

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13
Q

What disease is this autoantibody found and what does it test for?

c-ANCA

A
  • Disease
    • Active GPA (Wegener’s) >90%
  • Tests for:
    • Specific and sensitive
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14
Q

What disease is this autoantibody found and what does it test for?

p-ANCA

A
  • Disease
    • GPA (Wegener’s) 10%
    • Other vasculitis
  • Test for:
    • Nonspecific and poor sensitivity (found in ulcerative colitis, PAN, microscopic polyangiitis, Churg-Strauss, rapidly progressive glomerulonephritis)
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15
Q

What disease is this autoantibody found and what does it test for?

Anti-Mi-2

A
  • Disease
    • dermatomyositis 15-20%
  • Tests for:
    • Specific but not sensitive (not available in all centers)
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16
Q

What disease is this autoantibody found and what does it test for?

Ab against RBCs, WBCs, or platelets

A
  • Disease
    • SLE
  • Tests for:
    • Perform direct Coomb’s test
    • Test Hb, reticulocyte, leukocyte and platelet count, antiplatelet Abs
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17
Q

What is the pathophysiology and some examples of type I (anaphylactic) hypersensitivity?

A
  • Formation of IgE → release of immunologic mediators from basophils/mast cells → diffuse inflammation
  • Asthma, allergic reaction
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18
Q

What is the pathophysiology and some examples of type II (cytotoxic) hypersensitivity?

A
  • Formation of Ab → deposit and bind to Ag on cell surface → phagocytosis or lysis of target cell.
  • Autoimmune hemolytic anemia, Goodpasture’s syndrome, Graves’ disease, pernicious anemia.
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19
Q

What is the pathophysiology and some examples of type III (immune complex) hypersensitivity?

A
  • Formation of Ag-Ab complexes → activate complement → attract
    inflammatory cells and release cytokines.
  • SLE, PAN, post-streptococcal glomerulonephritis, serum sickness
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20
Q

What is the pathophysiology and some examples of type IV (cell-mediated/delayed) hypersensitivity?

A
  • Release of cytokines by sensitised T-cells and T-cell mediated cytotoxicity
  • Contact dermatitis
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21
Q

What are some patterns of joint involvement in disease?

A
  • Symmetrical vs. asymmetrical
  • Small vs. large
  • Mono vs. oligo (2-4 joints) vs. polyarticular (≥5 joints)
  • Axial vs. peripheral
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22
Q

What are the DDx of monoarthritis?

A
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23
Q

What are the DDx of oligoarhtritis/polyarthritis?

A
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24
Q

What are the symptoms of inflammatory arthritis vs degernative arthritis?

A
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25
Q

What are the differences between seropositive vs seronegative rheumatic disease?

Demographics, peripheral arthritis, pelvic/axial disease, enthesitis, extra-articular.

A
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26
Q

Define osteoarthritis.

A

progressive deterioration of cartilage and bone due to failed repair of joint damage caused by stresses on the joint

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27
Q

What the primary and secondary causes of osteoarthritis?

A
  • primary (idiopathic)
    • most common, unknown etiology
  • secondary
    • post-traumatic or mechanical
    • post-inflammatory (e.g. RA) or post-infectious
    • heritable skeletal disorders (e.g. scoliosis)
    • endocrine disorders (e.g. acromegaly, hyperparathyroidism, hypothyroidism)
    • metabolic disorders (e.g. gout, pseudogout, hemochromatosis, Wilson’s disease, ochronosis)
    • neuropathic (e.g. Charcot joints)
      • atypical joint trauma due to peripheral neuropathy (e.g. DM, syphilis)
    • AVN
    • other (e.g. congenital malformation)
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28
Q

Describe the pathophysiology of osteoarthritis?

A
  • deterioration of articular cartilage due to local biomechanical factors, which leads to joint trauma and release of proteolytic and collagenolytic enzymes
    • OA develops when cartilage catabolism > synthesis
    • loss of proteoglycans and water exposes underlying bone
  • abnormal local bone metabolism further damages joint
  • altered joint function and damage
  • synovitis is secondary to cartilage damage; therefore, may see small effusions in OA
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29
Q

Describe the epidemiology of osteoarthritis?

A
  • most common arthropathy
  • increased prevalence with increasing age (35% of 30 yr olds, 85% of 80 yr olds)
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30
Q

What are the risk factors for OA?

A
  • genetic predisposition
  • advanced age
  • obesity (for knee OA)
  • female
  • trauma
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31
Q

What are the signs and symptoms of OA?

A
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32
Q

What are the common joints involved in OA?

A
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33
Q

What are the common hand findings of OA?

A
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34
Q

What Ix need to be done for OA?

A
  • blood work
    • normal FBC and ESR, CRP
    • negative RF and ANA
  • radiology: 4 hallmark findings
  • synovial fluid: non-inflammatory
35
Q

What are the 4 radiographic hallmark signs of OA?

A
  1. Joint space narrowing
  2. Subchondral sclerosis
  3. Subchondral cysts
  4. Osteophytes
36
Q

What is the Rx for OA?

A
  • presently no treatment alters the natural history of OA
  • non-pharmacological therapy
    • weight loss (minimum 5-10 lb loss) if overweight
    • physiotherapy: heat/cold, low impact exercise programs
    • occupational therapy: aids, splints, cane, walker, bracing
  • pharmacological therapy
    • oral: acetaminophen/NSAIDs, glucosamine ± chondroitin (nutraceuticals not proven)
    • joint injections: corticosteroid, hyaluronic acid (questionable benefit)
      ƒ topical: capsaicin, NSAIDs
  • surgical treatment
    • joint debridement, osteotomy, total and/or partial joint replacement, fusion
37
Q

Define rheumatoid arthritis.

A
  • chronic, symmetric, erosive synovitis of peripheral joints (e.g. wrists, MCPs, MTPs)
  • characterized by a number of extra-articular features
38
Q

Describe the pathophysiology of RA?

A
  • autoimmune disorder, unknown etiology
  • hallmark of RA is hypertrophy of the synovial membrane
    • activated rheumatoid synovium (pannus) grows into and over the articular surface; inflammatory mediators lead to release of metalloproteinases and collagenases resulting in
      destruction of articular cartilage and subchondral bone
  • two theories attempt to explain chronic remissions and exacerbations seen in RA
    • sequestered Ag
      • during inflammation, immune complexes (ICs) are deposited at avascular cartilage-bone junction → immune complexes are released as further cartilage breaks down → triggers
        inflammatory cascade
    • molecular mimicry
      • cartilage damage → altered cartilage resembles undefined offending agent → triggers inflammatory cascade
39
Q

Describe the epidemiology of RA.

A
  • prevalence 1% of adult population
  • F:M = 3:1
  • age of onset 20-40 yr
  • genetic predisposition: HLA-DR4/DR1 association (93% of patients have either HLA type)
40
Q

Describe the signs and symptoms of RA.

Joint specific.

A
  • variable course of exacerbations and remissions
  • morning stiffness >1 h, improves with use, increases with rest
  • may have joint pain with activity
  • symmetric joint involvement
  • joint swelling, tender joints
  • constitutional symptoms: profound fatigue; rarely myalgia or weight loss
  • extra-articular features (EAF)
  • limitation of function and decrease in global functional status
  • complications of chronic synovitis
    • signs of mechanical joint damage: loss of motion, instability, deformity, crepitus, joint deformities
      • swan neck deformity, boutonnière deformity
      • ulnar deviation of MCP, radial deviation of wrist joint
      • hammer toe, mallet toe, claw toe
      • flexion contractures
    • atlanto-axial and subaxial subluxation
      • C-spine instability
      • neurological impingement (long tract signs)
      • difficult/dangerous intubation: risk of worsening subluxation and damage to spinal cord
    • limited shoulder mobility, spontaneous tears of the rotator cuff leading to chronic spasm
    • tenosynovitis → may cause rupture of tendons
    • carpal tunnel syndrome
    • ruptured Baker’s cyst (outpouching of synovium behind the knee); presentation similar to acute DVT
41
Q

What are the extra-articular manifestations of RA?

A
42
Q

What are the common sites of joint involvement in RA?

A
43
Q

What are the types of joint deformaties seen in RA?

A
44
Q

What are some syndromes associated with RA?

A
  • Srögren’s Syndrome (common): keratoconjunctivitis, sicca and xerostomia (dry eyes and mouth)
  • Caplan’s syndrome (rare): multiple pulmonary nodules and pneumoconiosis
  • Felty’s syndrome (rare): arthritis, splenomegaly, neutropenia
45
Q

What Ix need to be ordered for RA?

A
  • blood work
    • RF sensitivity ~80% but non-specific; may not be present at onset of symptoms
    • anti-CCP: sensitivity ~80% but more specific; may precede onset of symptoms
    • increased disease activity is associated with decreased Hb (anemia of chronic disease), increased platelets, ESR, CRP, and RF
  • imaging
    • x-rays may be entirely normal at onset
    • first change is periarticular osteopenia, followed by erosions
    • U/S, MRI may be used to image hands to detect early synovitis and erosions
46
Q

What are the poor prognostic features of RA?

A
  • young age of onset
  • high RF titer
  • elevated ESR
  • activity of >20 joints
  • presence of extra-articular features
47
Q

What are the goals of therapy for RA?

A
  • goals of therapy: remission or lowest possible disease activity
    • control disease activity
    • relieve pain and stiffness
    • Maintain function and lifestyle
    • ƒprevent or control joint damage
    • key is early diagnosis and early intervention with DMARDs
    • “window of opportunity” = early treatment within first 3 mo of disease may allow better control/remission
48
Q

What are the non-pharmacological Rx options for RA?

A
  • Education
  • Behavioural
    • exercise program (isometrics and active, gentle ROM exercise during flares, aquatic/aerobic/strengthening exercise between flares), assistive devices as needed
    • job modification may be necessary
49
Q

What are the pharmacological Rx for RA?

A
  • DMARDs and Biologics
    • DMARDs: standard of care and should be started as soon as possible
    • treatments guided by disease severity and prognostic features
    • MTX is the gold standard and is first-line unless contraindicated
    • delayed onset of action (may take 8-12 wk)
    • potential toxicities: GI, hematologic, hepatic (liver enzymes), pulmonary, teratogenic
    • if inadequate response (3-6 mo) → combine or switch
    • add-ons include: hydroxychloroquine, sulfasalazine, leflunomide
    • biologics: indicated if inadequate response to DMARDs
      • can be combined with DMARD therapy
      • agents include infliximab, etanercept, adalimumab, abatacept, rituximab, tocilizumab
      • reassess every 3-6 mo and monitor disease severity
  • Reducing Inflammation and Pain
    • NSAIDs
      • individualize according to efficacy and tolerability
      • contraindicated or cautioned in some patients (e.g. PUD, ischemic cardiac disease, pregnancy); add acetaminophen ± opioid prn for synergistic pain control
    • corticosteroids
      • local
        • IA injections to control symptoms in a specific joint
      • systemic (prednisone)
        • low dose (5-10 mg/d) useful for short-term to improve symptoms if NSAIDs ineffective, to bridge gap until DMARD takes effect
        • for severe RA, low dose prednisone can be added to DMARDs
        • do baseline DEXA bone density scan and consider bone supportive pharmacologic therapy if using corticosteroids >3 mo at >7.5 mg/d
        • cautions/contraindications: active infection, TB, osteoporosis, HTN, gastric ulcer, DM
50
Q

What are the side effects of steroids?

A
  • Weight gain
  • Osteoporosis
  • AVN
  • Cataracts, glaucoma
  • PUD
  • Susceptibility to infection
  • Easy bruising
  • Acne
  • HTN
  • Hyperlipidemia
  • Hypokalemia, hyperglycemia
  • Mood swings
51
Q

What are the surgical Rx options for RA?

A
  • indicated for structural joint damage
  • surgical options include: synovectomy, joint replacement, joint fusion, reconstruction/tendon repair
52
Q

What is the diagnostic criteria for SLE?

MD SOAP BRAIN

A
  • Malar rash
  • Discoid rash
  • Serositis
  • Oral ulcers
  • ANA
  • Photosensitivity
  • Blood
  • Renal
  • Arthritis
  • Immune
  • Neurologic
53
Q

Define SLE.

A
  • chronic inflammatory multi-system disease of unknown etiology
  • characterized by production of autoantibodies and diverse clinical manifestations
54
Q

What is the diagnostic criteria for SLE?

A
55
Q

Describe the aetiology and pathophysiology of SLE.

A
  • production of autoantibodies causing multi-organ inflammation
  • multi-factorial aetiology
  • genetics
    • common association with HLA-B8/DR3; ~10% have positive FHx
  • oestrogen
    • pre-pubertal and post-menopausal women have similar incidence to men
    • men with SLE have higher concentration of oestrogenic metabolites
  • infection
    • viral (non-specific stimulant of immune response)
  • drug-induced
    • anti-hypertensives (hydralazine), anti-convulsants (phenytoin), anti-arrhythmics (procainamide), isoniazid, biologics, OCPs
    • anti-histone Ab are commonly seen in drug-induced SLE
    • symptoms resolve with discontinuation of offending drug
56
Q

DDx “flu-like symptoms”.

A
  • Viral:
    • Influenza
    • HIV
    • CMV
    • Hepatitis A/B/C/D/E
    • Chicken pox
  • Bacterial:
    • Pneumonia
    • Syphilis
    • TB
  • Malignancy:
    • Leukemias/lymphoma
    • Hodgkins/non-Hodgkins lymphoma
  • Drugs:
    • Substance withdrawal
      • IgA nephropathy
  • Autoimmune
    • Reiters syndrome
    • Temporal arteritis
57
Q

Describe the epidemiology of SLE.

A
  • prevalence: 0.05% overall
  • F:M = 10:1
  • age of onset in reproductive yr (13-40)
  • more common and severe in African Americans and Asians
  • bimodal mortality pattern
    • early (within 2 yr)
      • active SLE, active nephritis, infection secondary to steroid use
    • late (>10 yr)
      • inactive SLE, inactive nephritis, atherosclerosis likely due to chronic inflammation
58
Q

What are the symptoms of SLE?

A
59
Q

What Ix can be done in SLE?

A
  • blood work: ANA (sensitivity 98%, but poor specificity → used as a screening test, ANA titres are not useful to follow disease course)
    • anti-dsDNA and anti-Sm are specific (95-99%)
    • anti-dsDNA titer and serum complement (C3, C4) are useful to monitor treatment response in patients who are clinically and serologically concordant
      • anti-dsDNA increases and C3 and C4 decrease with disease activity
    • antiphospholipid Ab (anti-cardiolipin Ab and SLE anticoagulant), may cause increased risk of clotting and increased aPTT
60
Q

Describe Raynaud’s Phenomenom?

A
  • Vasospastic disorder characteristically causing discoloration of fingers and toes (white → blue → red)
  • Classic triggers: cold and emotional stress
61
Q

What are the Rx goals of SLE?

A
  • treat early and avoid long-term steroid use, if unavoidable
  • if high doses of steroids necessary for long-term control, add steroid-sparing agents and taper when possible
  • treatment is tailored to organ system involved and severity of disease
  • all medications used to treat SLE require periodic monitoring for potential toxicity
62
Q

What are the Rx options for SLE?
Specifically dermatologic, MSK, and organ-threatening disease.

A
  • dermatologic
    • sunscreen, avoid UV light and estrogens
    • topical steroids, hydroxychloroquine
  • musculoskeletal
    • NSAIDs ± gastroprotective agent for arthritis (also beneficial for pleuritis and pericarditis)
    • hydroxychloroquine improves long-term control and prevents flares
    • bisphosphonates, calcium, vitamin D to combat osteoporosis
  • organ-threatening disease
    • high-dose oral prednisone or IV methylprednisolone in severe disease
    • steroid-sparing agents: azathioprine, MTX, mycophenolate
    • IV cyclophosphamide for serious organ involvement (e.g. cerebritis or SLE nephritis)
63
Q

What is CREST syndrome?

HINT: CREST

A
  • Calcinosis: calcium deposits on skin
  • Raynaud’s phenomenon
  • Esophageal dysfunction: acid reflux
  • Sclerodactyly: tightening of skin on digits
  • Telangiectasia: superficial dilated blood vessels
64
Q

Define gout.

A
  • derangement in purine metabolism resulting in hyperuricemia; monosodium urate crystal deposits in tissues (tophi) and synovium (microtophi)
65
Q

Describe the aetiology and pathogenesis of gout?

A
  • sources of uric acid: diet and endogenous
  • synthesis
    • hypoxanthine → xanthine → uric acid both steps catalyzed by xanthine oxidase
66
Q

What are the primary and secondary casues of hyperuricaemia?

A
  • primary or genetic
    • mostly due to idiopathic renal underexcretion (90%)
    • also idiopathic overproduction or abnormal enzyme production/function
  • secondary
    • dietary excess (particularly high consumption of beer, seafood, and meat)
    • underexcretion (>90%): renal failure, drugs, systemic conditions
    • overproduction (<10%): increased nucleic acid turnover states (e.g. malignancy, post-chemotherapy)
  • sudden changes (increasing or decreasing) in uric acid concentration are more important than absolute values
    • acute gout can occur with normal serum uric acid
    • changes in pH, temperature, or initiation of antihyperuricemics may precipitate an acute gouty attack
  • common precipitants: alcohol, dietary excess, dehydration, drugs (e.g. thiazide and loop diuretics), trauma, illness, surgery, starting xanthine oxidase inhibitor therapy
  • other associated conditions: HTN, obesity, DM, starvation
67
Q

What are the signs and symptoms of gout?

A
  • single episode progressing to recurrent episodes of acute inflammatory arthritis
  • acute gouty arthritis
    • severe pain, redness, joint swelling, usually involving lower extremities
    • joint mobility may be limited
    • attack will subside spontaneously within several days to weeks; may recur
  • tophi
    • urate deposits on cartilage, tendons, bursae, soft tissues, and synovial membranes
    • common sites: first MTP, ear helix, olecranon bursae, tendon insertions (common in Achilles tendon)
  • kidney
    • gouty nephropathy
    • uric acid calculi
68
Q

What Ix need to be done for gout?

A
  • joint aspirate: >90% of joint aspirates show crystals of monosodium urate
    (negatively birefringent, needle-shaped)
  • x-rays may show tophi as soft tissue swelling, punched-out lesions – erosion with “overhanging” edge
69
Q

What is the Rx for gout?

Acute

A
  • NSAIDs: high dose, then taper as symptoms improve
  • corticosteroids: IA, oral, or intra-muscular (if renal, cardiovascular, or GI disease and/or if NSAIDs contraindicated or failed)
  • colchicine within first 12 h but effectiveness limited by narrow therapeutic range
  • allopurinol can worsen an acute attack (do not start during acute flare)
70
Q

What is the Rx for gout?

Chronic

A
  • conservative
    • avoid foods with high purine content (e.g. visceral meats, sardines, shellfish, beans, peas)
    • avoid drugs with hyperuricemic effects (e.g. pyrazinamide, ethambutol, thiazide, alcohol)
  • medical
    • antihyperuricemic drugs (allopurinol and febuxostat): decrease uric acid production by inhibiting xanthine oxidase
    • uricosuric drugs (probenecid, sulfinpyrazone): use if failure on or intolerant to allopurinol; do not use in renal failure
  • prophylaxis prior to starting antihyperuricemic drugs (colchicine/low-dose NSAID)
  • in renal disease secondary to hyperuricemia, use low-dose allopurinol and monitor Cr
  • indications for treatment with antihyperuricemic medications include:
    • recurrent attacks, tophi, bone erosions, urate kidney stones
    • perhaps in renal dysfunction with very high urate load (controversial)
71
Q

What are the common sites of involvement in gout?

A
72
Q

What are the side effects of allopurinol?

A
  • Skin rash - maculopapular
  • Allopurinol hypersenitivity syndrome: fatal, erythematous desquamating rash, fever, hepatitis, eosinophilia, and worsening renal function.
73
Q

Define antiphospholipid antibody syndrome, and the causes?

A
  • multi-system vasculopathy manifested by recurrent thromboembolic events, spontaneous abortions, and thrombocytopenia
  • often presents with migraine-type headaches
  • circulating antiphospholipid autoantibodies interfere with coagulation cascade
  • primary APLA: occurs in the absence of other disease
  • secondary APLA: occurs in the setting of a connective tissue disease (including SLE), malignancy, drugs (hydralazine, procainamide, phenytoin, interferon, quinidine), and infections (HIV, TB, hepatitis C, infectious mononucleosis)
  • catastrophic APLA: development within 1 wk of small vessel thrombotic occlusion in ≥3 organ systems with positive antiphospholipid Ab (high mortality)
74
Q

How does antiphospholipid antibody syndrome manifest?

A
  • Thromboembolic events: Artherial and venous thrombosis are usually mutually exclusive.
    • Renal vein thrombosis
    • Glomerular thrombosis
    • MI
    • TIAs
  • Spontaneous abortions
  • Thrombocytopenia, haemolytic anaemia, neutropenia
  • Dermatologic
    • livedo reticularis
    • Raynaud’s phenomenon
    • pupura
    • leg ulcers
    • gangrene
  • Migrane headaches
  • Addison’s disease
  • Epilepsy
75
Q

What is the Rx for antiphospholipid antibody syndrome?

A
  • thrombosis
    • lifelong anti-coagulation with warfarin
    • target INR 2.0-3.0 for first venous event, >3.0 for recurrent and/or arterial event
  • recurrent fetal loss
    • heparin/low molecular weight heparin ± Aspirin® during pregnancy
  • catastrophic APLA
    • high-dose steroids, anti-coagulation, cyclophosphamide, plasmapheresis
76
Q

Define adult onset stills disease?

A
  • systemic inflammatory condition (ANA and RF negative) with fevers and characteristic rash, numerous systemic symptoms, and may have severe arthritis
77
Q

What is the aetiology of Adult onset Stills disease?

A
  • idiopathic; infectious triggers likely – various viruses and bacteria have been implicated
  • stress increases risk
78
Q

What is the classic triad for Stil’s disease?

HINT: FAR

A
  • Fever
  • Arthralgias/Arthritis
  • Rash
79
Q

What are the signs and symptoms of Adult onset Stills disease?

A
  • classic triad of symptoms
    • high-spiking fevers (95.7% of patients, typically T = 102.2ºF/39°C, <4 h duration, quotidian pattern)
    • characteristic “salmon rash” (~72% of patients, on proximal limbs + trunk)
    • arthralgia/arthritis (64-100%)
  • sore throat, myalgias and serositis may also occur
  • arthritis is symmetric, typically affects large joints, i.e. wrists, knees and ankles, may involve PIP and DIPs, elbow, MTPs
  • liver abnormalities ± hepatomegaly (50-75% patients)
  • splenomegaly (44%)
80
Q

What Ix need to be done with suspected adult onset Stills disease?

A
  • ANA and RF negative
  • markedly elevated ESR, CRP, ferritin (typically >1000 ng/mL, >2200 pmol/L)ƒ total ferritin >5x ULN = 80% sensitive, 41% specific
  • anemia, thrombocytosis, leukocytosis may occur
  • transaminases, LDH may be elevated
81
Q

What is the Rx for adult onset stills?

A
  • refer to rheumatologist for treatment with biologics (anti-IL1 and anti-IL6 agents)
  • begin management with low-dose glucocorticoids ± MTX
82
Q

What are the radiological features of rheumatoid arthritis?

A
  • Joint space narrowing
  • periarticular osteoporosis
  • Erosions
  • Periarticular soft tissue swellinig
  • Later joint destruction or subluxation
83
Q

Behçet’s syndrome is characterised by?

A
  • Recurrent oral and genital ulcers
  • Uveitis
  • Seronegative arthritis
  • Central nervous system symptoms
  • Fever
  • Thrombophlebitis
  • Erythema nodosum
  • Abdominal symptoms, and
  • Vasculitis.

Year 4 (25 decks)

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