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342 Cards in this Set

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DR4, DR1



Sjogren's syndrome, SLE, RA, Celiacs, T1DM, Grave's

List causes of polyarticular involvement

Infectious: lyme disease, bacterial endocarditis, gonococcus, viral (EBV, parvovirus)

Post-infectious: Rheumatic fever, reactive arthritis, enteric infections

Inflammatory: RA, SLE, Seronegatives

List causes of monoarticular involvement








ESR is thought to indicate the amount of____ in the blood


What is the physiological role of C-reactive protein

bind to lysophosphatidylcholine expressed on the surface of dead or dying cells inorder to activate the completment systemic by C1Q complex

Where is CRP made?

Liver, in response to factors released by macrophages and adipocytes

How did CRP get it's name?

It was first identified in serum of patients with acute inflammation by its reaction to C-polysaccharide of pneumococcus

The acute phase response is initiated (predominately) by...

increased concentrations of IL-6 produced by macrophages

What is the half-life of CRP


Which is a better measure of response to treatment: ESR or CRP

CRP returns to normal more quickly than ESR in response to therapy

List postive acute phase reactants

CRP, mannose-binding protein, complement factors, ferritin, cerulosplasmin, haptoglobin, PT, fibrinogen, FVII, vWF

List negative acute phase reactants

albumin, transferrin, retinol-binding protein, antithrombin, transcortin


mucosa: gut, resp tract, urogenital tract.

present in saliva, tears, breast mile


B-cell receptor. Acitvates basophils, mast cells


binds to allergens and triggers histamine

What is the only antibody capable of crossing the placenta?


What antibody forms a pentamer?

IgM (secreted form)

On what chromosome are HLA antigens encoded?


What component of an antibody determines it isotype?

Heavy chain (contant region)

What are the 2 types of light chains?

kappa and lambda

What is rheumatoid factor?

an antibody against Fc of IgG

What % of healthy individuals have rheumatoid factor?

5-10%; increases with age

Rheumatoid Factor is present in what % of patients with RA?

80% (70% in Sjorgrens)

RF is associated with what viral illnesses?

parvovirus, EBV

What autoantibody correlates with disease activity in SLE?



Sjorgren's 40%, SLE 25%


Sjorgren's 40%, SLE 10%

What autoantibody is present in >80% of patients with CREST?



active Wegener's >90%


Wegners 10% (non specific, poor sensitivity)

low C3 serum levels are associated with...

formation of immune complexes e.g. post-infectious glomerulonephritis, SLE

What are components of synovial fluid

ultrafiltrate of plasma + hyaluronate

What is the origin of synovial fluid?

synovial membrane (Type B cells)

List 3 functions of synovial fluid?

reduction of friction, shock absorption, nutrient and waste transportation

what is normal glucose concentration in synovial fluid?

same as serum

Crystals present in gout

monosodium urate (needle shaped negatively briefringent crystals 2-20microm)

Crystals present in pseduogout

calcium pyrophosphate

Needle shaped negatively-birefringent crystals in synovial fluid

gout (monosodium urate)

Rod shaped/rhomboids with positive birefringence

pseudogout (calcium pyrophosphate)

yellow in parallel light, blue with perpendicular light

negative birefringence (gout)

blue with parallel light, yellow with perpendicular ligth

positive birefringence (pseudogout)

Describe appearance of corticosteroid crystals?

blunt, jagged, variable birefringence

Synovial fluid is a dilatant liquid. What does that mean?

viscosity increases with rate of shear strain- acts as shock absorber

Define negatively birefringent

yellow when parallel to acis of red compensator

Radiological findings in inflammatory arthritis

periarticular osteopaenia, erosions, uniform decrease in joint space

Radiological findings in non-inflammatory arthritis

local cartilage loss, irregularly decreased joint space, bony overgrowth, cyst formation

Loss of proteoglycans and water from synovial fluid is key pathogenesis in ...


radiological finding in osteoarthritis

subchondral sclerosis, uneven narrowing of joint space, osteophytis

Most common joints affected by OA


Hand deformities associated with osteoarthritis

DIP (heberden's nodes), PIP (Bouchard's nodes). MCP often spared

Etiology of pseudogout

increased ATP breakdown with resultant increased inorganic pyrophosphate in joints from aging, genetic factors

Osteoarthritis of the MCP joint is associated with what conditions?

haemochromatosis, chrondocalcinosis

Which movements tend to be lost first in hip OA?

internal rotation and abduction of hip

Most common cause of sciatica?

disc protrusion or posterior osteophytes

Neurological claudication is a sign of....

spinal stenosis

4 classic radiological findings in osteoarthritis

joint space narrowing, osteophytes, intraosseous cyst, osteophytes

How does subchonrdal sclerosis appear on xray

mottled, increased opacity just adjacent to joint space

Clinical features of scleroderma

Raynaud's, stiffness of fingers, ski tightness, heartburn/dysphagia

Skin tightness on dorsum of hand, facial skin tightening, telangiectasia, calcinosis, non-effusive joint


False positive VDRL can occur in...


Incidence of RA

2 per 1,000

Genetic pre-disposition of RA


What is the pathological hallmark of rheumatoid arthritis

pannus: hypertrophy of synovical membrane with growth of granulation tissue

List the Dx criteria of RA

morning stiffness >1hr for >6wks

3 or more joints >6wks

Arthritis in at least 1 of: MCP, PIP, wrist for >6wks

Symmetric arthritis for >6wks

Rheumatoid nodules

Serum RF

Xray changes

Xray changes in RA

erosions or periarticular ostepenia, joint effusions.

Joint deformities in RA

Swan neck, boutonniere, ulna deviation, hammer toes, flexion contractures, atlanto-axial subluxation

Baker's cyst

outpouching of synovium behind the knee

swan neck

hyperextension of PIP, flexion of DIP


fixed flexion contracture of PIP, extended DIP


bone marrow suppression, liver disease, immunodeficiency

Entanercept and infliximab are...

TNF inhibitors

Side effects of corticosteriods

osteoporosis, avascular necrosis, HTN, cataracts, glaucoma, peptic ulcer, psychosis, susceptibility to infection, hypokalaemia, hyperglycaemia, hyperlipidemia

peripheral polyarthritis with symmetric involvement of small and large joints WITHOUT joint erosion


Dx criteria of SLE

1. Malar rash

2. Discoid rash

3. Photosensitivity

4. Oral/nasal ulcers (painless)

5. Arthritis

6. Serositis: pleurisy, pericarditis, peritonitis

7. Neurologic disorde: headahce, seizures

8. Nephropathy

9. Leukopaenia, hemolytic anaemia

10. Autoantibodies dsDNA, Sm

11. ANA

Serologic hallmark of SLE

high titre ANA

lupus anticoagulant has what effect on clotting

tendency to bleed (increases APTT)

what is antiphospholipid antibody syndrome (APS)

multisystem vasculopathy manifested by recurrent thromboembolic, spontaneous abortions, thrombocytoaenia

Clinical features of antiphospholipid antibody syndrome

VTE, renal/retinal thrombosis, stroke, TIA, multiinfarct dementia, chorea, MI, limb ischaemia, recurrent miscarriages, livedo reticularis

What are dermatological features of antiphospholipid antibody syndrome

livedo reticularis, perpura

What is Dx criteria of antiphospholipid antibody syndrome

lupus anticoagulant or anticardiolipid antibody positive on 2 occasions, at least 8wks apart.


calcinosis, raynaud's, esophageal dysmotility, sclerodactyly, telangiectasia

Characteristic facial features in CREST

mask-like facies, beak nose, radial perioral furrows, pigment changes, matt telangiectasia, pruritis

What is Raynaud's phenomenon

episodes of blanching and/or cyanosis of digits followed by erythema, tingling and pain due to vasospasm

what is antiphospholipid antibody syndrome

mutlisystem vasculopathy manifested by recurrent thromboembolic events, spontaneous abortions and thrombocytopaenia

pathogenesis of systemic sclerosis

intimal proliferation and medial mucinous degeneration, progresive obliteration of vessel lumen, decreased vascular luminal size, secondary fibrosis of tissues

Describe the involvement of GI tract in systemic sclerosis (90%)

hypomotility, sphincter dysfunction

List 3 types of cartilage?

elastic, hyaline, fibrocartilage


reduced proliferation of chondrocytes in epiphyseal plate of long bones during infancy and childhood (dwarfism)

Inclusion body myositis

slowly progressive symmetrical proximal muscle weakness (shoulder and hip) that develops over wks to mnths with increase in muscle enzyme levels

What are pathonomonic of dematomyositis

Gottron's papules, Gottron's sign

What is Gottron's sign

symmetrical, erythematous, scaly eruption over MCP and IP joints. Associated with Inclusion Body Myositis

Diagnostic features of inclusion body myositis

- progressive symmetric proximal muscle weakness

- muscle enzyme levels: increased CF, aldolase, LDH, transaminases (AST, ALT)

- EMG: short polyphasic motor units, high frequency repetitive discharge, insertional irritability

- Muscle biospy: segmental fibre necrosis, basophilic regeneration, perivascular inflammation and atrophy

chronic, inflammatory disorder characterized by CD4/CD8 cell-mediated infiltration and destruction of salivary and lacrimal glands

sjogren's syndrome

Anti-cholinergic side effects

constipation, xerostomia, mydriasis, blurred vision, urinary hesitancy, reduced GI motility, anticholinergic derlirium

Dx of Sjogren's (SSASSS)

Schirmer test (assess tear flow)

Slit lamp exam with Rose-Bengal stain

Autoantibodies (anti-ro and -la)

Salivary flow measurement


Salivary gland biopsy: gold standard

Dx of sjogren's

2 of the following: characteristic salivary biopsy, keratoconjunctivitis sicca, associated connective tissue or lymphoproliferative disorder

ANCA-associated vasculitis

Wegener's granulomatosis

Churg-Strauss vasculitis

Microscopic polyangiitis

Medium-sized vessel vascultitis

polyarteritis nodosa, kawasaki's

Large vessel vasculitis

giant cell arteritis (temporal arteritis)


NonANCA associated small vessel disease

predominantly cutaneous vasculitis

Henoch Schonlein purpura

Essention cryoglobulinemic vasculitis


Henoch Schonlein purpura. Vascular deposition of IgA causing systemic vasculitis (skin, GI, renal). usually self limiting

Granulomatous inflammation of small and medium sized artieries and veins of respiratory tract and kidneys

wegener's granulomatosis

Most common presentation of Wegener's granulomatosis

URTI symptoms

Clinical feature of wegener's granulomatosis

systemic: malaise, fever, weakness, weight loss

respiratory: upper tract: sinusitis, rhinitis, nasoseptal perforation, saddle nose deformity, otitis media, hemoptysis, tracheobronchial erosin, pneumonitis, cavity formation

Kidney: segmental necrotizing glomerulonephritis

Diagnosis of Wegener's granulomatous

>2 of

- nasal or oral inflammation

- abnormal findings on CXR, including nodules, cavitations

- Urinary sediment

- Biopsy of involved tissue: lungs show granulomas, kidneys show necrotising segmental glomerulonephritis

Small and medium vasculitis + eosinophilia

Churg strauss syndrome

alergic rhinitis, asthma, systemic vasculitis

churg strauss syndrome

focal panmural necrotising inflammatory lesions in small and medium sized arteries

polyarteritis nodosa

Which vasculitis is associated with hypertension and aneurysmal dilatation of kidney vessels

polyarteritis nodosa

inflammation of medium and large sized arteries, predominantly those originating from the aortic arch and the aorta itself.

temporal arteritis

Clinical features of giant cell arteritis

over 50 yrs, more common in women

temporal headaches and scalp tenderness

Vasculitis associated with sudden, painless loss of vision, diplopia

temporal arteritis

vasculitis associated with Polymyalgia rheumatica

Temporal arteritis

Buerger's disease

thromboangiitis obliterans. Inflammation is secondary to pathological clotting

Ocular involvement, recurrent oral and genital ulceration, venous thrombosis, skin and joint involvement

Behcet's disease

leukocytoclastic venulitis

Behcet's disease

Reactive arthritis generally develops how long after the initial infection

1-4wks post infection

Sausage digits "dactylitis" are characteristic of ...

reactive and psoriatic arthritis

Psoriasis affects what % of population?


In serological results, titre e.g. 1:40 refers to....

the dilution at which the Ab become undetectable. Higher titre = more antibodies

Anti-centromere pattern of ANAs is suggestive of...

CREST (calcinosis, Raynaud's, eosphageal dysmotility, sclerodactyly, telangiectasia)

Anti-ds DNA (in higher titre) and Anti-Smith ENA are very specific for ...


List drugs associated with drug-induced lupus

Procainamide, hydralazine, chlorpromazine, isoniazid, sulfasalazine, methyldopa, quinidine, minocycline, anti-TNF therapy.

Anti-topoisomerase I are also known as...? What are they specific for?

Anti-Scl-70; systemic sclerosis (present in 75% of cases, associated with progressive skin involvement, pulmonary fibrosis and higher mortality)

Which antibodies are associated with congenital heart heart block?

Anti-Ro/SSA and La/SSB

Anti-U1-RNP is a very sensitive indicator for which rheumatologic disorder?

Mixed connective tissue disorder

c-ANCA and pANCA are associated with what epitopes respectively?

c-ANCA- Proteinase 3 PR3

p-ANCA- myeloperoxidase (MPO)

The combination of c-ANCA and anti-PR3 is specific for which condition?

Wegener's (granulomatosis with polyangiitis)

What is the most common cause of anti-MPO + drug induced ANCA associated vasculitis

propylthiouricil, methimazole

List conditions that are both p-ANCA and anti-MPO positive?

MPA ,EGPA, Churg Strauss, pauci-immune RPGN, Anti-GBM, drug induced vasculitis

List conditions that are pANCA + but anti-MPO-

Crohns, UC, PSC, PBC, chronic arthritides

C4 is consumed only with activation of which complement pathway?

Classical e.g. SLE

C3 is consumed with activation of which complement pathways

Both classical and alternative

What is rheumatoid factor?

An auto-antibody that binds to the Fc region of IGG. Positive in 80% of patients with RA

What is the most specific test for FA?

Anti-citrullinated cyclic peptide (anti-CCP); 97% specficity

Does FR+ anti-CCP confer positive or negative prognosis?

negative - more aggressive, more extraarticular manifestations

Which bacteria may cause a false positive HLA-B27 test

Klebsiella pneumonia

What % of patients of the caucasian population has HLA-B27

7-8% of healthy population

What % of patients with Ankylosing spondylitis have the HLA-B27 gene


Class I HLA molecules interact with which T cells

CD8 or T suppressor

Class II HLA molecules interact with which T cells

CD4 or T helper

What % of patients with reactive arthritis have HLA-B27

60-70%; higher when sacroiliitis is present

If axial arthropathy is present, which gene is most likely present?


For an individual carrying the HLA-B27 gene, what its he risk they will develop related disease?


A negative HLA-B27 test is useful in ruling out which condition?

ankylosing spondylitis

Which HLA genes are associated with SLE

DR2 and DR3

Which HLA gene is associated with severe RA


Which HLA is associated with Behcet disease? What is Behcet disease?

HLA-B51; rare immune-mediated small vessel systemic vasculitis presenting with recurrent oral pathos ulcers, genital ulcers and uveitis. Vascular aneurysms

Other than RA, a positive rheumatoid factor can be seen in what other disease?

SLE, Sjorgren's, infective endocarditis, TB, HIV

Which cells make cartilage?


Which crystal are implicated in gout?

monosodium urate crystals

What cystals are implicated in pseudo gout?

calcium pryophosphate

What is meant by negatively birefringent

crystals that are yellow when parallel to the compensator e.g uric acid

What is meant by positive birefringent?

crystals that are blue when parallel to compensator e.g. pseudogout

Synovial fluid microscopy shows rhomboid positive birefringent crystals

pseudogout/calcium pyrophosphate

Synovial fluid microscopy shows needle-like negative birefringent crystals

gout/uric acid

List features of Marfan syndrome

long limbs, pacts excavatum or pacts carinatum, aortic aneurysm/dissection, ectopic lentis, valvular disease

What are the different types of Ehlers-Danlos

Type II and II- easily scarred skin and hypermobile

Vacular type- predominantly skin involvement, predilection for rupture of large vessels

List features of osteogenesis imperfecta

osteopenia, multiple fractures, blue sclera, lucent brittle teeth, hearing loss

What is pseudoxanthoma elasticum

AR disorder; mutation in ABCC6 gene; involving skin papules and laxity, retinal peau d'orange leading to angled streaks and retinal haemorrhages

What is the prevalence of RA? what is the male:female ratio?

0.5%-1%; 1:3

What is the specificity of anti-CPP for Dx of RA?


List factors which indicate poor prognosis in RA?

Presence of HLA-DR4, higher titre RF or anti-CCP antibodies, elevated acute phase reactants, >6 joints involves, constitutional symptoms, radiographic evidence of erosive disease, extraarticlar disease e.g. nodules, vasculitis, serositis

In RA, which joints of the hands are affected?

MCP and PIP; DIP is spared

Which hand deformities classically occur in RA

Boutonniere and swan neck

Summarise the diagnostic criteria for RA

Inflammatory arthritis 1-10 joints, RF and/or anti-CPP, Increased ESR or CRP, duration >6wks

Hoarseness in a patient with RA may suggest...

involvement of the cricoarytenoid joint

What are the cardiac manifestations of RA?

Pericarditis/myocarditis, nodules on valves, 3 x increased risk of sudden death and MI

What is the leading cause of death among patients with RA?

coronary artery disease

What is Felty Syndrome

RA, splenomegaly, neutropaenia (spleen can be felt-y)

What are common side effects of methotrexate

alopecia, GI upset, BM suppression, increased LFTs. Less common: severe hepatotoxicity, nephrotoxicity, pneumonitis/pulmonary fibrosis)

Does the dose of methotrexate need to be adjusted in the context of AKI?


Which DMARD used to treat RA classically causes retinopathy?

hydroxychloroquine 1:5,000

Is methotrexate induced pneumonitis dose related or idiosyncratic?


Which infections may become reactivated with methotrexate

Hep B and Tb

Prior to commencing azathioprine, the activity of which enzymes need to be checked? Why>

TPMT (Thiopurine methyltransferase); 1 in 300 people have low or no detectable activity which increases risk of myelosuppression.

6-TGN high rbc concentrations of 6-TGN are associated with increased efficacy/myelotoxicity.

6-MMP high rbi concentrations of 6-MMP increased risk of hepatoxocitiy

How does allopurinol affect azithioprine

increased serum level and potential toxicity of azathioprine

What is the mechanism of action of cyclosporine?

calcinruin inhibitor; binds to cyclophilin and blocks action of calcineurin in T cells to prevent production of IL-2 and other cytokines

What are adverse effects of cyclosporine

Gingival hyperplasia, nephrotoxicity, HTN, hypercholesterolaemia, raised LFTs, hypomagnesaemia, hyperkalaemia, diarrhoea, hyperglycaemia, diabetes, tremor, headache

Describe the joint involvement in SLE?

inflammatory, non-erosive, may be symmetrical or asymmetrical, often involves small joints of hands and wrist

What is the most common cause of death in patients with SLE


What are the patients of skin rash seen in SLE

photosensitive butterfly/malar (25%)

Subacute cutaneous lupus erythematosis 5%

Discoid lupus 5%

What % of patients with SLE have cutaneous involvement?


How can you differentiate rosacea from SLE malar rash?

Malar rash is photosensitive, spares nasobalial folds, and may also involve neck/chest/chin. Rosacea is characterised by telangiectasis, pustules and papules without comedones

What % of patients with primary disoid lupus develop SLE?


What % of patients with SLE develop Raynauds phenomenon


What is the most common form of lung involvement in SLE?

pleuritic chest pain +/- effusions

What is Libman-Sack endocarditis?

sterile fibro-fibinous vegetations that can mimic infectious endocarditis; commonly associated with anti-phospholipid Ab

Which autoantibodies in SLE are associated with CNS disease?

Anti-Smith, anti-neuronal and anti-ribosomal P Ab

List ACR 1997 diagnostic criteria for SLE? (SOAP BRAIN MD)


Oral ulcers (usually painless)

Arthritis (non-erosive_


Blood disorders

Renal disease


Immunological Ab (dsDNA, anti-Sm, anticardiolipin)


Malar rash

Discoid rash

Which test is 99% sensitive for SLE


What is HELLP?

Preeclampsia with homeless, elevated LFTs, platelets

Foetuses of mothers with SLE who have SSA/Ro and SSB/La abs are at risk of what conditions from the 2nd trimester onwards? How is this prevented?

Heart block; treatment with hydroxychloroqine decreases risk

Up to 1/3 of SLE patients on chronic high-dose glucocovrticosteroids develop what bone condition?

avascular necrosis of hip/knee/humerus

What medications improve survival in patients with SLE and class III or IV GN?

cyclophosphamide and corticosterods

List factors that carry a negative prognosis in SLE?

High anti-dsDNA, low complements levels (increased risk for nephritis)

What drugs classical cause drug-induced lupus?

procainamide, hydralazine, chlorpromazine, PTU, phenytoin, TNF inhibitors

Which Abs are usually positive in drug induced lupus?

ANA, Anti-histone

Sausage digit + pitted nails =

psoriatic arthritis

What is the concordance between identical twins for ankylosing spondylitis


List extraarticular manifestations of ankylosing spondylitis

Iritis/uveitis, conjunctivitis, IHD, aortic insufficiency/aortitis, apical pulmonary fibrosis, IgA nephropathy

How does iritis or uveitis present?

unilateral pain, photophobia, increased lacrimation

Which organisms are associated with reactive arthritis?

chlamydia trachomatis, salmonella, shigella, yersinia, campylobacter, c. diff, HIV

What % of patients with IBD develop associated arthropathy


What is the classic triad of findings seen in reactive arthritis

urethritis, conjunctivitis, asymmetric oligoarthritis

What % of patients with isolated skin psoriasis develop arthritis?

7%; compared to 20-30% of patients with nail involvement?

Describe classic skin features of psoriasis?

salmon-coloured plaques on extensor surfaces

Which drug, sometimes used in treatment of arthritis, might exacerbate the psoriatic rash?

TNF inhibitors

Penicl-in-cip DIP appearance on X-ray suggests what Dx?

psoriatic arthritis (as opposed to Gull-wing seen in OA)

Why should corticosteroids be avoided in psoriatic arthritis?

withdrawal can lead to severe pustular psoriasis

Bouchards deformity affects what joint?


Heberden deformity affects what joint


Hallux valgus

knock knees

Hallux varus

bow legged

List causes of decreased renal excretion of uric acid?

Idiopathic or secondary to CKD, lead, alcohol, drugs (aspirin, thiazide/loops, calcineurin inhibitors), diabetic ketoacidosis

List causes of increased production of uric acid

idiopathic, leukaemia, haemolytic anaemia, tumor lysis syndrome, psoriasis, exercise, fructose ingestion, G6PD deficiency

How is gout definitively diagnosed?

joint aspirate that shows intracellular need-shaped negative birefringent crystals consistent with monosodium urate

What is the uric acid concentration target in a patient who has more that 1 attack of gout a year?

<600mg/l which is below the saturation of monosodium urate

With which drugs do you have to dose adjust (decrease) allopurinol?

azathioprine, mercaptopurine, theophylline

What is the mechanism of action of allopurinol?

xanthine oxidase inhibitor; reduces production of uric acid

50yo patient presents with 2 day history of fever blistering mucosa and rash. blood tests show and AKI, eosinophilia, and deranged LFTs. PMHx includes gout for which he takes allopurinol. He has no allergies. Dx?

TEN/SJS secondary to allopurinol

Which HLA is associated with allopurinol-realted TEN/SKS


What is uricase?

An enzyme that oxidatively degrades uric acid to soluble allantoin that can be readily excreted via the urine. Absent in humans.

What is the mechanism of action of probenecid?

increases renal excretion of uric acid by blocking its renal tubular reabsorption. Reduces renal tubular excretion of some acidic drugs (e.g. penicillins) increasing their plasma concentrations and prolonging durationg of action.

Describe the effect of aspirin on uric acid excretion

low dose prevents excretion,high dose causes uricosuria.

What causes chondrocalcinosis

calcium pyrophosphate cyrstal depositive

List factors which predipose patients to chondrocalcinosis

primary hyperparathyroidism, haemochromotasis, hypothyroiism, hypomagnesemia, hypophosphataemia

Rhomboid, positive birefringence (light blue when parallel), chondrocalcinosis suggests...


What % of the population has psoriasis?


List antiphospholipid Ab?

lupus anticoagulant, anti-cardiolipin, beta2glycoprotein

How do you dx antiphospholipid syndrome

One or more antiphospholipid Abs on 2 occasionsat least 12 wks apart + either thrombosis or miscarriage/premature births

List features of antiphospholipid syndrome

one or more antiphopholipid Abs on 2 occasions at least 12 wks apart + ether thrombosis or miscarriage/premature births; livedo reticular, sterile cardiac vegetation(Libman-Sacks endocarditis), thrombocytopaenia, prolonged APTT)

What is the association between B cell lymphoma and Sjorgren syndrome?

40x increased risk


dry mouth


dry eyes

How do you confirm a diagnosis of Sjorgren's disease?

Autoantibodies, biospy a salivary gland

What is the Schemer test? What is positive?

To dx dry eyes. <5mm of wetting in 5mnths. Normal is 15mm

Do steroids improve sick symptoms in Sjogrens


What % of patients with systemic sclerosis have Scl-70 Ab?

30%; associated with reduced survival and interstitial lung disease

Anti topoisomerase I ab are also known as


What are the implications of anti-RNA polymerase III in systemic scerosis

much higher risk of renal crisis, lower risk of ILD when compared to anti-Scl-70

What is CREST

calcinosis, raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia

What % of patients with CREST have anti-centromere Ab? Is it specific?

Present in 50% (low sensitivity) but high specificity

What is the most common pulmonary disease in CREST

pulmonary hypertension (ILD unlikely)

Describe the pathological progression of skin changes seen in scleroderma?

mucinous edema, induration, fibrosis, atrophy

Describe nailfold findings seen via capillaroscopy in systemic sclerosis

capillaries are reduced in number, remaining vessels become giant loops. Correlates with severity of SSc

Describe the joint involvement in systemic sclerosis

mild, systemic hand stiffness +/- synovitis. Tendon friction rub (pathognomonic)

What is the main cause of morbidity and mortality in systemic sclerosis

lung disease: pulmonary arterial HTN, interstiital fibrosis

How can systemic sclerosis cause pulmonary HTN without interstitial lung disease

PAH: intimal proliferation

ILD: interstital fibrosis secondary to alveolitis and fibrosis

Abnormal nailfold capillaries are more commony seen in which autoimmune disease

Systemic sclerosis; fewer large capillary loops

How does renal crisis present in systemic sclerosis

presents within first 5 yrs of diffuse disease with malignant HTN, AKI and acute urine sediment. Associated with prior or current steroid use and presence of anti-RNA polymerase III Ab

What is the mainstay of treatment to prevent renal crisis in systemic sclerosis


What is GAVE

watermelon stomach; gastric antral vascular ectasia. Associated with CREST

What medications change the course of scleroderma

sadly, none

Wide mouthed diverticular of the GI tract are pathognomonic of which systemic disease?

systemic sclerosis

T or F: the extent of skin disease in systemic sclerosis is a marker of the severity of visceral disease


T or F: myositis usually presents with muscle weakness not pain


What is Gottrons sign

erythematous scaly rash over MCP and PIP

List drugs which commonly cause myopathy?

alcohol, colchicine, statins

What is the prognosis of Jo-1 associated myositis

70% 5yr survivial `

What is a heliotrope rash

violaceous, appears on upper eyelids

What is the most specific clinical findings in dermatomyositis?

Gottron papules (reddish scaly papules over the MCP and PIPs)

What is antisynthetase syndrome

A specific presentation of dermatomyositis/polymyositis characterised by very acute onset disease, fever, weightless, joint and often lung involvement. Associated with Anti-Jo-1 (type of antisynthase antibodies)

What are EMG findings in myositis?

increased fibrillations, decreased amplitude, spontaneous repetitive activity with early recruitment.

What is Trendelenburg's sign

positive if when standing on one leg the pelvis drops on the opposite side to the standing leg. Weakness in hip abductors e.g. glut medius or minimus

What is the gold standard Dx test in myositis

muscle biopsy

List antibodies associated with myositis

Anti-Jo-1, Anti-M202, Anti-SRP, Antip 155/p140

A patient with dermatomyositis + anti-p155/140 Ab have what risk of cancer?


Which is the risk of cancer highest following Dx of polymyositis or dermatomyositis?

in the first 5 yrs

True or false. Every patient who is newly Dxwith either polymyositis or dematomyositis must be evaluated for underlyingmalignancy with total body PET/CT?

False. Should do detailed history and exam andage appropriate screening tests. Not full body imaging

What are the most common malignancies associatedwith polymyositis or dematomyositis must

Ovarian, lung, pancreatic, colon, lymphoma

How are polymyositis or dematomyositis treated?

High dose prednisione that is slowly taperedwhile a steroid sparing agent is added (e.g. MTX or azathioprine)

What is the response rate to corticosteroids inpolymyositis or dematomyositis?

80% begin to respond within a few days to 6weeks

A patient with polymyositis initially improveson corticosteroids but then develops progressive weakness despite improvementin CK levels. DDx?

Superimposed steroid myopathy- Reassess for malignancy

What is the most common inflammatory myopathy inpeople >50yrs old? Is it more common in men or women?

Inclusion body myositis; more common inCaucasian men

60yo man present with new, gradual onset distalweakness. You not he has a weak handshake. Dx?

inclusion body myositis

True or false. CK is markedly elevated in inclusion body myositis?

False. May be only mildly elevated.

What is seen on muscle biopsy in inclusion bodymyositis?

Vacuoles and filamentous inclusions

Is inclusion body myositis responsive tosteroids?

Minimally responsive

What is the most common cause of myopathy?

statins and chronic corticosteroids

Which drugs are used to treat fibromyalgia?

Antidepressants +/- anti-convulsants

What are the clinical features of serotoninsyndrome?

Clonus, hyperreflexia, tachycardia, sweating,hyperthermia, flushing

65 yo woman with fibromyalgia for which shetakes tramadol and paroxetine presents with sweating and tachycardia. On examshe has clonus and hyperreflexia. Dx?

Serotonin syndrome

Which drugs can cause serotonin syndrome

SSRIs, SNRIs, tramadol, MAOI, sumatriptan, TCAs,LSD, St Johns Wort, Amphetamines, Lithium

What are the most common ANCA-associated vasculitides

granulomatosis with polyangiitis, microscopic polyangiitis, churg strauss

List large vessel vasculitides

Giant cell arteritis, takayasu

Describe the pathology of giant cell arteritis

Multinucleated giant cells infiltrate bloodvessels arising from the aortic arch in a patchy or segmental fashion

What disease presents with temporal headache,diploplia, amaurosis fugax, scalp tenderness, jaw claudication?

Giant cell arteritis

Does a negative temporal artery biopsy excludeGCA?

No, due to patchy involvement

What % of PMR patients develop GCA?


Is CK elevated in PMR?


Is ESR elevated in PMR?

Yes, >50mm/hr (normal in 5%)

Takayasu arteritis commonly effects what demographic

asian women <40

List the 4 most common medium/small arteryvasculities

Polyarteritis nodosa, churg-strauss, Wegner(granulomatosis with polyangiitis), microscopic polyangiitis

Which medium/small artery vasculitis is NOTassociated with ANCA?


Describe the pathological process in PAN

Accumulation of neutrophils and mononuclearcells within the arterial walls with fibrinoin necrosis and subsequentformation of aneurysms. Not granulomatous.

Classic PAN commonly affects the arteries thatsupply the skin, peripheral nerves, GI tract and kidney but spares the ______


With which virus is PAN associated?

Hep B

How does the treatment of PMR differ from GCA?

PMR – low dose steroidsGCA – high dose steroids

Does PAN cause GN?


What is the 5yr survival of PAN if untreated?


What is the treatment of PAN?

Treat chronic HBV

Prednisone +/- cyclophosphamide

Name the disease characterized by necrotizing,pulmonary-renal vasculitis marked by eosinophilic granulomas?

Churg Strauss

>10% peripheral eosinophilia, pANCA+,anti-MPO with a background of asthma suggests

Churg Strauss

What is the hallmark of Wegners?

Necrotizing granulomas in small vessels; c-ANCA+ and anti-PR3

Saddle nose deformity is associated with which vasculitis?


List side effect of cyclophosphamide?

Bone marrow suppression, sterility, amenorrhea, hemorrhagic cystitis, loss of/metallic taste, heart failure, pulmonary fibrosis, water retention, secondary malignancies 3-8yrs after treatment, immunosuppression e.g. PJP

What is the major dose-limiting factor in treatment with cyclophosphamide?

myelosuppresion; neutrophil nadir occurs 7-14days after dose, recovers 1-2wks later

Name an IgA mediated mediated small vessel vasculitis that can affect arterioles, capillaries and venues?

Henoch Schonlein Pupura

What type of vessels does HSP affect

small vessel vasculitis that can affect arterioles, capillaries and venules

HSP affects which organs

small vessels of skin (waist down, palpable purpura), kidney, GI tract, joints

What is the prognosis of HSP

Most cases are self-limited; 20% have a repeat attack, 5% develop chronic HSP

Describe the 3 types of cryoglobulins

Type I: least common (10-15%), single monoclonal Ab e.g. Waldenstrom,multiple myeloma. Complement not activated -> asymptomatic untilhyperviscosity developsType II: most common 50-60%. Immune complexes consisting of monoclonal IgM rheumatoid factorattached to polyclonal IgG. Hep C.Type III: 25-30% polyclonal IgM RF andpolyclonal IgG. Hep C, SLE, lymphoproliferative malignancy.

Low C4 out of proportion to C3 suggests


Which cryoglobulins activate compliment? Which have RF?

Type II, III

What % of patients with anti-GBM (Goodpasture's) also have ANCA? What is the implication of this?

15%; symptoms of systemic vasculitis

Describe Behcet disease? Which people are most likely to be effected?

Painful recurrent venous ulcers (genital, ocular, skin). Middle east/Asian 20-30s

What complications can be seen in Behcets?

interstinal ulceration, mesenteric vasculitis, blindness, meningoencephalitis, GN palsies, thrombosis or rupture of aneurysms

What is the end produce of purine metabolism in humans?

uric acid

T or F: in bursitis, pain is far worse with active movement. Pass ROM is less painful or even painless.


Name the 4 bursae of the knee

Suprapatellar, infrapatellar, prepatellar, pes anserine

Where is the peso anserine bursa?

~2cm inferiorly and medial to patella. Pes anserinus is the tendons of sartorial, graceless, semitensdinosis that insert into the tibia.

Adhesive capsulitis is colloquially known as...

Frozen shoulder

What are the 4 muscles of the rotator cuff?

teres minor, supraspinatus, infraspinatus, subscapularis

65yo man presents with lateral shoulder and armpain. He has been waking with the pain at night. On exam, the middle arc ofactive abduction is painful, while the extremes are painless. There istenderness to palpation over the lateral shoulder. Dx?

Subacromial bursitis DDx supraspinatustendonitis/calcification

Olecranon bursistis is associated with whichsystemic disease?


Tennis elbow is more formally known as...

Lateral epicondylitis; pain and tendernesslocalized to the front of the lateral epicondyle of the elbow where theextensor tendons of the forearm insert

Golfer’s elbow is more formally known as…

medial epicondylitis

What is the most common cause of lateral thighdiscomfort?

trochanteric bursitis

Which patients are at risk for AVN?

Chronic glucocorticoid use, alcoholics,pregnancy, sickle cells disease, HIV/AIDs, Gaucher disease, hypercoaguablestates, pancreatitis, IBD and SLE. Femoral neck fractures, hip dislocations.

Which sign is seen on MRI of hip AVN?

Crescent sign signifying subcondral collapse

Where is the most common site of pain in hip OA?


Xray features of OA?

Joint space narrowing subchondral sclerosisosteophytes

What is a Baker cyst?

Posterior herniation of synovial cavity behindthe knee caused by a tense knee effusion.

50 yo woman presents with burning pain andtenderness between the 3rd and 4th toes of her rightfoot. She states “it feels like I’m standing on a pebble all the time”.Palpation of the interspace produces sharp pain radiating to the toes.Squeezing the forefoot ilicits the same senations. Dx?

Morton (Plantar) Neuroma

List red flags of back pain

Known cancer Dx, multiple risk factors forcancer, risk of osteomyelitis (e.g. IVDU, TB exposure, immunosuppression),urinary retention, foecal incontinence, progressive motor weakness

Disc herniation is most common at whichvertebral level?

L55/S1 because of progressive thinning of theposterior longitudinal ligament

T or F: Classic disk herniation pain is worsewhen sitting or bending and better when lying or standing.


What is spondyloysis?

Defect in the isthmus of the nueral arch (parsinterarticularis) of the 5th (rarely the 4th) lumbarvertebra

Name another disease that must be considered ina patient with plantar fasiitis?

Ankylosing spondylitis

What is spondylolisthesis?

A spontaneous subluxation (usually forward) ofone lumbar vertebra over another (usually anterior subluxation of L4 over L5)