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342 Cards in this Set
- Front
- Back
DR4, DR1 |
RA |
|
DR3 |
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 |
Infection Crystalopathy Haemarthrosis Tumour Trauma Inflammatory Degenerative |
|
ESR is thought to indicate the amount of____ in the blood |
fibrinogen |
|
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 |
48hrs |
|
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 |
|
IgA |
mucosa: gut, resp tract, urogenital tract. present in saliva, tears, breast mile |
|
IgD |
B-cell receptor. Acitvates basophils, mast cells |
|
IgE |
binds to allergens and triggers histamine |
|
What is the only antibody capable of crossing the placenta? |
IgG |
|
What antibody forms a pentamer? |
IgM (secreted form) |
|
On what chromosome are HLA antigens encoded? |
6 |
|
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? |
anti-dsDNA |
|
Anti-RO |
Sjorgren's 40%, SLE 25% |
|
Anti-La |
Sjorgren's 40%, SLE 10% |
|
What autoantibody is present in >80% of patients with CREST? |
anti-centromere |
|
c-ANCA |
active Wegener's >90% |
|
p-ANCA |
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 ... |
osteoarthritis |
|
radiological finding in osteoarthritis |
subchondral sclerosis, uneven narrowing of joint space, osteophytis |
|
Most common joints affected by OA |
knee>hip>hand>spine |
|
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 |
scleroderma |
|
False positive VDRL can occur in... |
SLE |
|
Incidence of RA |
2 per 1,000 |
|
Genetic pre-disposition of RA |
HLA DR4/DR1 |
|
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 |
|
boutonnieres |
fixed flexion contracture of PIP, extended DIP |
|
Methotrexate |
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 |
SLE |
|
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. |
|
CREST |
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 |
|
Achondroplasia |
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 Sialography 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) Takayasu's |
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NonANCA associated small vessel disease |
predominantly cutaneous vasculitis Henoch Schonlein purpura Essention cryoglobulinemic vasculitis |
|
HSP |
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? |
1% |
|
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 ... |
SLE |
|
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 |
90% |
|
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? |
HLA-B27 |
|
For an individual carrying the HLA-B27 gene, what its he risk they will develop related disease? |
10-20% |
|
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 |
DR4 |
|
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? |
Chondrocytes |
|
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? |
97% |
|
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? |
Yes |
|
Which DMARD used to treat RA classically causes retinopathy? |
hydroxychloroquine 1:5,000 |
|
Is methotrexate induced pneumonitis dose related or idiosyncratic? |
indiosyncratic |
|
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 |
CVD |
|
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? |
80% |
|
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? |
10% |
|
What % of patients with SLE develop Raynauds phenomenon |
30% |
|
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) |
Serositis Oral ulcers (usually painless) Arthritis (non-erosive_ Photosensitivity Blood disorders Renal disease ANAs Immunological Ab (dsDNA, anti-Sm, anticardiolipin) Neurologic Malar rash Discoid rash |
|
Which test is 99% sensitive for SLE |
ANAs |
|
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 |
60% |
|
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 |
20% |
|
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? |
PIP
|
|
Heberden deformity affects what joint
|
DIP |
|
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 |
HLA-B*5801 |
|
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... |
pseudogout |
|
What % of the population has psoriasis? |
~3% |
|
List antiphospholipid Ab? |
lupus anticoagulant, anti-cardiolipin, beta2glycoprotein |
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How do you dx antiphospholipid syndrome |
One or more antiphospholipid Abs on 2 occasionsat least 12 wks apart + either thrombosis or miscarriage/premature births |
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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) |
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What is the association between B cell lymphoma and Sjorgren syndrome? |
40x increased risk |
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Xerostomia |
dry mouth |
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Xerophthamlia |
dry eyes |
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How do you confirm a diagnosis of Sjorgren's disease? |
Autoantibodies, biospy a salivary gland |
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What is the Schemer test? What is positive? |
To dx dry eyes. <5mm of wetting in 5mnths. Normal is 15mm |
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Do steroids improve sick symptoms in Sjogrens |
No |
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What % of patients with systemic sclerosis have Scl-70 Ab? |
30%; associated with reduced survival and interstitial lung disease |
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Anti topoisomerase I ab are also known as
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Scl-70 |
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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 |
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What is CREST |
calcinosis, raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia |
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What % of patients with CREST have anti-centromere Ab? Is it specific? |
Present in 50% (low sensitivity) but high specificity |
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What is the most common pulmonary disease in CREST |
pulmonary hypertension (ILD unlikely) |
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Describe the pathological progression of skin changes seen in scleroderma? |
mucinous edema, induration, fibrosis, atrophy |
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Describe nailfold findings seen via capillaroscopy in systemic sclerosis |
capillaries are reduced in number, remaining vessels become giant loops. Correlates with severity of SSc |
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Describe the joint involvement in systemic sclerosis |
mild, systemic hand stiffness +/- synovitis. Tendon friction rub (pathognomonic) |
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What is the main cause of morbidity and mortality in systemic sclerosis |
lung disease: pulmonary arterial HTN, interstiital fibrosis |
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How can systemic sclerosis cause pulmonary HTN without interstitial lung disease |
PAH: intimal proliferation ILD: interstital fibrosis secondary to alveolitis and fibrosis |
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Abnormal nailfold capillaries are more commony seen in which autoimmune disease |
Systemic sclerosis; fewer large capillary loops |
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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 |
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What is the mainstay of treatment to prevent renal crisis in systemic sclerosis
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ACEi |
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What is GAVE |
watermelon stomach; gastric antral vascular ectasia. Associated with CREST |
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What medications change the course of scleroderma |
sadly, none |
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Wide mouthed diverticular of the GI tract are pathognomonic of which systemic disease? |
systemic sclerosis |
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T or F: the extent of skin disease in systemic sclerosis is a marker of the severity of visceral disease
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True |
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T or F: myositis usually presents with muscle weakness not pain |
true |
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What is Gottrons sign
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erythematous scaly rash over MCP and PIP |
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List drugs which commonly cause myopathy? |
alcohol, colchicine, statins |
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What is the prognosis of Jo-1 associated myositis |
70% 5yr survivial ` |
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What is a heliotrope rash |
violaceous, appears on upper eyelids |
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What is the most specific clinical findings in dermatomyositis? |
Gottron papules (reddish scaly papules over the MCP and PIPs) |
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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) |
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What are EMG findings in myositis? |
increased fibrillations, decreased amplitude, spontaneous repetitive activity with early recruitment. |
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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 |
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What is the gold standard Dx test in myositis |
muscle biopsy |
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List antibodies associated with myositis
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Anti-Jo-1, Anti-M202, Anti-SRP, Antip 155/p140 |
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A patient with dermatomyositis + anti-p155/140 Ab have what risk of cancer? |
70% |
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Which is the risk of cancer highest following Dx of polymyositis or dermatomyositis? |
in the first 5 yrs |
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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 |
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What are the most common malignancies associatedwith polymyositis or dematomyositis must |
Ovarian, lung, pancreatic, colon, lymphoma |
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How are polymyositis or dematomyositis treated? |
High dose prednisione that is slowly taperedwhile a steroid sparing agent is added (e.g. MTX or azathioprine) |
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What is the response rate to corticosteroids inpolymyositis or dematomyositis? |
80% begin to respond within a few days to 6weeks |
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A patient with polymyositis initially improveson corticosteroids but then develops progressive weakness despite improvementin CK levels. DDx? |
Superimposed steroid myopathy- Reassess for malignancy |
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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 |
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60yo man present with new, gradual onset distalweakness. You not he has a weak handshake. Dx? |
inclusion body myositis |
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True or false. CK is markedly elevated in inclusion body myositis? |
False. May be only mildly elevated. |
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What is seen on muscle biopsy in inclusion bodymyositis? |
Vacuoles and filamentous inclusions |
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Is inclusion body myositis responsive tosteroids? |
Minimally responsive |
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What is the most common cause of myopathy? |
statins and chronic corticosteroids |
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Which drugs are used to treat fibromyalgia? |
Antidepressants +/- anti-convulsants |
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What are the clinical features of serotoninsyndrome? |
Clonus, hyperreflexia, tachycardia, sweating,hyperthermia, flushing |
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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 |
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Which drugs can cause serotonin syndrome |
SSRIs, SNRIs, tramadol, MAOI, sumatriptan, TCAs,LSD, St Johns Wort, Amphetamines, Lithium |
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What are the most common ANCA-associated vasculitides |
granulomatosis with polyangiitis, microscopic polyangiitis, churg strauss |
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List large vessel vasculitides |
Giant cell arteritis, takayasu |
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Describe the pathology of giant cell arteritis |
Multinucleated giant cells infiltrate bloodvessels arising from the aortic arch in a patchy or segmental fashion |
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What disease presents with temporal headache,diploplia, amaurosis fugax, scalp tenderness, jaw claudication? |
Giant cell arteritis |
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Does a negative temporal artery biopsy excludeGCA? |
No, due to patchy involvement |
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What % of PMR patients develop GCA? |
20% |
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Is CK elevated in PMR? |
No |
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Is ESR elevated in PMR? |
Yes, >50mm/hr (normal in 5%) |
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Takayasu arteritis commonly effects what demographic |
asian women <40 |
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List the 4 most common medium/small arteryvasculities |
Polyarteritis nodosa, churg-strauss, Wegner(granulomatosis with polyangiitis), microscopic polyangiitis |
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Which medium/small artery vasculitis is NOTassociated with ANCA? |
PAN |
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Describe the pathological process in PAN |
Accumulation of neutrophils and mononuclearcells within the arterial walls with fibrinoin necrosis and subsequentformation of aneurysms. Not granulomatous. |
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Classic PAN commonly affects the arteries thatsupply the skin, peripheral nerves, GI tract and kidney but spares the ______ |
lungs |
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With which virus is PAN associated? |
Hep B |
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How does the treatment of PMR differ from GCA? |
PMR – low dose steroidsGCA – high dose steroids |
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Does PAN cause GN? |
No |
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What is the 5yr survival of PAN if untreated? |
10-29% |
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What is the treatment of PAN?
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Treat chronic HBV Prednisone +/- cyclophosphamide |
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Name the disease characterized by necrotizing,pulmonary-renal vasculitis marked by eosinophilic granulomas?
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Churg Strauss |
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>10% peripheral eosinophilia, pANCA+,anti-MPO with a background of asthma suggests |
Churg Strauss |
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What is the hallmark of Wegners? |
Necrotizing granulomas in small vessels; c-ANCA+ and anti-PR3 |
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Saddle nose deformity is associated with which vasculitis? |
Wegeners |
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List side effect of cyclophosphamide?
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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 |
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What is the major dose-limiting factor in treatment with cyclophosphamide? |
myelosuppresion; neutrophil nadir occurs 7-14days after dose, recovers 1-2wks later |
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Name an IgA mediated mediated small vessel vasculitis that can affect arterioles, capillaries and venues?
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Henoch Schonlein Pupura |
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What type of vessels does HSP affect |
small vessel vasculitis that can affect arterioles, capillaries and venules |
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HSP affects which organs
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small vessels of skin (waist down, palpable purpura), kidney, GI tract, joints |
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What is the prognosis of HSP
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Most cases are self-limited; 20% have a repeat attack, 5% develop chronic HSP |
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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. |
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Low C4 out of proportion to C3 suggests |
cryoglobulin |
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Which cryoglobulins activate compliment? Which have RF?
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Type II, III |
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What % of patients with anti-GBM (Goodpasture's) also have ANCA? What is the implication of this? |
15%; symptoms of systemic vasculitis |
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Describe Behcet disease? Which people are most likely to be effected? |
Painful recurrent venous ulcers (genital, ocular, skin). Middle east/Asian 20-30s |
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What complications can be seen in Behcets? |
interstinal ulceration, mesenteric vasculitis, blindness, meningoencephalitis, GN palsies, thrombosis or rupture of aneurysms |
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What is the end produce of purine metabolism in humans?
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uric acid |
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T or F: in bursitis, pain is far worse with active movement. Pass ROM is less painful or even painless. |
True |
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Name the 4 bursae of the knee |
Suprapatellar, infrapatellar, prepatellar, pes anserine |
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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. |
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Adhesive capsulitis is colloquially known as... |
Frozen shoulder |
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What are the 4 muscles of the rotator cuff? |
teres minor, supraspinatus, infraspinatus, subscapularis |
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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 |
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Olecranon bursistis is associated with whichsystemic disease? |
RA |
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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 |
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Golfer’s elbow is more formally known as… |
medial epicondylitis |
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What is the most common cause of lateral thighdiscomfort? |
trochanteric bursitis |
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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. |
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Which sign is seen on MRI of hip AVN? |
Crescent sign signifying subcondral collapse |
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Where is the most common site of pain in hip OA? |
Groin |
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Xray features of OA? |
Joint space narrowing subchondral sclerosisosteophytes |
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What is a Baker cyst? |
Posterior herniation of synovial cavity behindthe knee caused by a tense knee effusion. |
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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 |
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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 |
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Disc herniation is most common at whichvertebral level? |
L55/S1 because of progressive thinning of theposterior longitudinal ligament |
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T or F: Classic disk herniation pain is worsewhen sitting or bending and better when lying or standing. |
True |
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What is spondyloysis? |
Defect in the isthmus of the nueral arch (parsinterarticularis) of the 5th (rarely the 4th) lumbarvertebra |
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Name another disease that must be considered ina patient with plantar fasiitis? |
Ankylosing spondylitis |
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What is spondylolisthesis? |
A spontaneous subluxation (usually forward) ofone lumbar vertebra over another (usually anterior subluxation of L4 over L5) |