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four types of SLE 1) spontaneous, 2) discoid (skin lesions w/o other involvement), 3) Rx induced, 4) ANA -
characteristics of ANA - SLE Ro (SS-A) +, has arthritis, Raynauds, subacute cutaneous Lupus, risk of neonatal Lupus
key features of neonatal Lupus born to Ro (SS-A) + women, skin lesions w cardiac anomalies incl heart block (AV), tGA, and valve/septal anomalies
3 MC sympt in Lupus malar rash (1/3), joint pain (90%), fatigue (common)
what steps in screening for Lupus ANA should be + (but not specific), then check dsDNA and Sm Ab; if ANA - should check Ro (SS-A) and La (SS-B) which is + in ANA - SLE
describe LE (lupus erythemosus) prep ANAs bind nuclei damaged cells creating LE bodies
name some of the other connective tissue dzs that can be ANA + RA, scleroderma, Sjorgens, polymyositis and dermatomyositis
describe features of SLE by organ system: mucocutaneous, CVS, pulmon, hemo, renal, GI mucocut: malar rash, discoid rash, photosensit, Reynauds, oral uclers; CVS: peri/myocarditis, Libman-Sacks endocarditis; pulmon: pleuritis (MC); heme: hemolytic anemia, decrsd WBC and plts, + lupus anticoag; renal: proteinuria, cell casts; GI: N/V
name all immunol/Ab abnml in SLE ANA, dsDNA & Sm Ab (specific), Ro & La (in ANA -, assoc neonatal SLE), anti His (Rx SLE), VDRL/RPR syph, anticardiolipin and lupus anticoag,
tx SLE NSAIDs, local or systemic steroids +/- hydroxychloroquine
name GN grading for SLE, which is MC and which assoc w renal failure I=min, II=mesangial, III=focal prolifer, IV=diffuse prolifer (40%, renal failure is common), V=membranous; note: GN usu present at dx
describe anti-phospholipid syndrome and what dzs it's assoc w recurrent arterial or venous thrombosis, recurrent fetal loss, decrsd plts, livedo reticularis (purplish lace-like rash on LE)
name HLAs assoc w SLE, Sjorgen, RA, ankylosing spondylitis, Reitiers, psoriatic arthritis SLE=DR2,3; Sjorgen=DR2; RA=DR4; ankylosing spondylitis, Reitiers, psoriatic arthritis=B27
what's the MC presenting sympt of scleroderma Reynauds
what dz is commonly found w Scleroderma Sjorgens (20%)
key Abs for scleroderma CREST or limited=anti-centromere; diffuse=anti-Scl70 (or topoisomerase?)
CREST stands for Calcinosis digits, raynauds, esophageal, sclerodactyly, telangiectasia (digits, nails)
describe skin involvement in scleroderma subtypes, how relate to dz progression in limited only extremities and head and neck are involved (not trunk), whereas diffuse has widespread skin involvemnet; amt skin involvement can predict dz
what need to monitor in scleroderma esophageal motility w Ba swallow and PFTs (MC cause of death is pulmon)
describe sympt unique to diffuse scleroderma ILD, peripheral edema, polyarteritis and carpal tunnel, fatigue, mscle involvement, + visceral involvement of lungs, heart, GI, kidney
describe pathophys of Sjorgen lymphocytes invade lacrimal and salivary glands
describe 2 types of Sjorgens primary w/o another connective tissue dz, 2ry w RA, sclero, SLE, polymyositis
what Ab + in Sjorgens in 50% Ro (SS-A) and La (SS-B) are + [not specific]; note: SS-A at risk for neonatal SLE
tx Sjorgens pilocarpine and eye drops
what Ab + in mixed connective tissue dz; describe the dz anti-UI-RNP; overlap of dzs but don't nec occur simultaneously
pts who present w RA usu women 20-40
describe arthritis and key radiographic findings of RA symmetrical inflamm polyarthritis w hot, swollen joints, MC PIP, MCP, wrists NOT DIP; radiographic: periarticular osteoporosis, bony erosion, pannus (cartilage is like granulomatous tissue) + ulnar deviation, swan neck, and boutenierre deformities
key findings accompany RA C1, C2 cervical instability, pl eff common, pericarditis, episcleritis, rheum nodules
kids w uveitis, inflamm arthritis, RF -…what dz? RA (often RF isn't + and in pauciarticular)
tx of RA; tx of OA tx RA=NSAIDs (pain) + combo of MTX, hydroxychloroquine, sulfsalazine (Dz modifying Rx); tx OA=acetaminophen (since no inflamm component, or NSAIDs but concern PUD with long term use) can give joint steroids
MC cause of arthritis osteoarthritis
key findings of OA on radiography joint space narrowing, osteophytes, sclerosis of subchondral bone, subchondral cysts; Bouchards=PIP and Heberdens DIP
name of genetic syndrome assoc w gout Lesch Nyhan defic in hypoxanthine guanidine phosphoryibosyltrxse
joint aspirate of gout v pseudogout v RA all will have WBC>5,000, gout has needle shaped - birefringent, pseudogout has wkly + rod/rhomboid
what rheum dz can MTX be used for, and what are SE; and what must you monitor RA; SE incl GI upset, oral ulcers, bone marrow suppression, hepatocell injury, interstitial pneumonitis **give folate and monitor LFTs and renal
tx acute gout NSAIDs (indomethacin) and colchicine [not ASA makes worse, not acetaminophin bc doesn't have anti-inflamm prop]
SE of colchicine GI (80%): N/V, abd cramps, severe diarrhea
prophylaxis for gout, when to give, what Rx give after 2 attacks, Rx: if <800mg/d urine uric acid then under secreting uric acid and give probenacid or sulfinpyrazone; if >800 then overproduction is the problem and give allopurinol
after gout attack what should you avoid to prevent more attacks thiazide and loop diuretcs (incrs uric acid in blood), EtOH, dietary purine [secrets says to alkanize urine and incrs fluids]
what type of crystals form in pseudo gout calcium pyrophosphate
MC organism causing septic arthritis; what does joint aspirate show S Aureus, but in young sexually active N Gonorrhea (which will have + cultue in only 1/4); joint aspirate will show >50,000 WBC and >70% PMN (v 5,000 WBC and 50-70%PMN in inflamm arthritis)
how is pseudogout treated esstly same as gout
clinical findings common to polymyositis and dermatomyositis symmetrical prox mscle wknss (trbl getting up from chair, climbing steps), myalgia (1/3), dysphagia (1/3)
what skin findings seen in dermatomyositis heliotrope rash, V sign, shawl sign, Gotton's papules=papular, eryth scaly lesions over knuckles
once dermatomyositis is dx what do you need to look for other malignancy
key markers and Abs in dermatomyositis/polymyositis CPK (mrkr dz severity), anti-synthetase (anti-Jo-1): abrupt onset, poor px; anti-signal recognition protein: cardiac, worst px; anti-Mi-2: best px
how is mscl bx different bw polymyositis and dermatomyositis polymyositis (and inclusion body)=endomysial inflamm and fibrosis; dermatomyositis=perivascular and perimysial
how is inclusion body myositis difft from polymyositis and dermatomyositis more common in elderly men, prox and distal wkness, no Abs, slight incrsd CPK, poor px and response to tx
who does polymyalgia rheumatica occur in, what else to look for? more elderly women, 10% have temporal arteritis
what are the muscles most often affects in polymyalgia rheumatica? neck, shoulder, pelvic girdle [also for myositis]
what is clinical dx for fibromylagia 11 of 18 points >3mos
what are key distinguishing features of fibormyalgia pain is constant, aching, aggravated by stress, cold, sleep deprivation and better w rest, warmth, and mild exercise; insomnia; anxiety depression
name the seronegative spondylarthropathies ankylosing spondylitis, reactive arthritis/Reiters, psoriatic arthritis
what are clinical findings of ankylosing spondylitis key is low back pain (bilateral sacroilitis) w limited motion, can have constitutional +/- uveitis
distinguishing features of reactive arthritis/Reitiers occurs after an enteric infxn (Salmonella, Shigella, Camp, Yersinia) or urethritis (ie Chlamydia); asymm arthritis progresses from 1 joint to another usu LE
classic Reiters characteristics arthritis, uveitis, urethritis after enteric or GU infxn +/- genital and oral ulcers like Behcets
name the large artery vasculitis Takayasu and temporal
name the med vessel arteritis (5) PAN, Kawasaki, Wegeners, Churg Strauss, microscopic polyangitis
name the small arteritis (3) HSP, hypersensitivity vasculitis, Behcets
key features temporal arteritis, what else to look for >50, women, new onset headache, temporal pain and jaw claudication; look for ophthalmic artery involvement and 40% hav polymyalgia rheumatica
key features of, dx, cxns of Takayasu young Asian women, involved Ao arch, pulseless; dx w arteriogram; cxns incl Ao anurysm and renal artery stenosis causing HTN
key features of, dx, tx of Churg Strauss palpable purpura in pt w asthma, Eos; dx by skin bx (Eos) and pANCA; tx: steroids but poor px
key features of, dx, tx of Wegeners hemoptysis, hematuria, bloody sinusitis; cANCA + (open lung bx confirms); tx=steroids and cyclophosph but poor px (death <1yr in most)
key features of, dx, tx of PAN assoc w Hep B, HIV, Rx rxns; can have bowel angina but no pul involvement; pANCA may be + but need bx for dx; steroids helps some; poor px
common present of Behcets young male 20s w painful oral and genital ulcers +/- uveitis, arthritis, eryth nodosum (but no prior infxn unlike Reiters)
dx, tx Behcets need bx, steroids
key features of, dx, tx of hypersensitivity vasculitis mostly skin involved w palpable purpura, macules, vesicles, usu LE; Rx ie PCN, sulfa or infxn; dx=bx; withdrawal agent and steroids
auto Ab: ANA SLE (but not specific)
auto Ab: DNA specific SLE
auto Ab: Smith specific SLE
auto Ab: His Rx induced Lupus
auto Ab: c-ANCA Wegeners
auto Ab: p-ANCA Churg Strauss, microscopic polyangitis
auto Ab: microsomal Hashimotos thyroiditis
auto Ab: centromere CREST scleroderma
auto Ab: Scl 70 diffuse scleroderma
auto Ab: mitochondrial primary biliary cirrhosis (scleroderma)
auto Ab: GBM Goodpasture (more specific Col IV)
auto Ab: IgG rheum arthrit
auto Ab: gliaden celia sprue (allergic to gluten)
which Ab specific for Lupus DNA and smith (His for Rx-induced Lupus)
HLAB27 assoc w PAIR psoriasis, ankyl spond, inflamm bowel, Reiters
name seroneg arthritis Reiters, ankyl spondyl, psioriatic arth (no rheum factor (anti IgG); all assoc HLA B27)
describe Reiters symptoms, cause can't see, pee, climb tree (conjunctivitis/uveitis, urethritis, arthritis), usu s/p Gi or chlamydia
CREST calcinosis, rayneud, esophag, sclerodactyly, telangiectesia
CREST v diffuse scleroderma, incl px diffuse: widespread skin involve, visceral involve, rapid progress (Scl-70 Abs); CREST more benign and limited (fingers and face)
name mech of 4 hypersensit I:preformed IgE on mast, leads to histamine rel; II preformed Ab, Ag on cell-> cell lysis; III Ag-Ab complex leads to complement activ; IV T cells sensitized to Ag
ACID pneu for hypersensit I A=anaphylactic, II C=cytotoxic, III I=immune complex, IV D=delayed
type I hypersensitivity incl anaphylaxis, allergic rhinitis
type II hypersensitivity incl hemolytic anemia, ITP, erythro blast fetalis, MG, Rheum F, Goodpasture, Graves, Bullous pemph
type III hypersensitivity incl post-strep glomerulo, polyarteritis nodosum, RA, SLE
type IV hypersensitivity incl MS, Guillan Barre, granulomas, PPD, contact dermatitis, DM type I
difft cholangitis and cholecystitis cholangitis=bile duct, cholecystitis=gall bladder
what hypersensit type is SLE? III
what hypersensit type is Hashimoto? IV
what hypersensit type is Graves? II
what hypersensit type is MS? MG? MS=IV, MG=II
what hypersensit type is polyarteritis nodosa? III
what hypersensit type is hyperacute transfusion? II
what hypersensit type is hemo anemia? II
what hypersensit type is ITP? II
describe MS symptoms SIN scan speech, intention tremor/INO/incontinence, nystagmus; sudden vision loss, paresthesia, periventricular plaques
describe MG symptoms ptosis, diplopia, general PROX muscle weakness; symptoms get worse w muscle use
tx MS beta interferon or immunosuppress
MG mech auto Ab ag Ach R
dx, tx MG dx: edrophonium, tx: pyridostigmine or neostigmine (AChE inhibitor)
what dz can appear like MG Lambert-Eaton syn (in small cell lung cancer); auto Ab to presynapt Ca channels leads to decrs Ach rel; but sympt imprv w mscl use
Created by: ehstephns
 

 



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