Connect Tiss USMLE
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| four types of SLE | 1) spontaneous, 2) discoid (skin lesions w/o other involvement), 3) Rx induced, 4) ANA -
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| characteristics of ANA - SLE | Ro (SS-A) +, has arthritis, Raynauds, subacute cutaneous Lupus, risk of neonatal Lupus
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| 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
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| 3 MC sympt in Lupus | malar rash (1/3), joint pain (90%), fatigue (common)
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| 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
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| describe LE (lupus erythemosus) prep | ANAs bind nuclei damaged cells creating LE bodies
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| name some of the other connective tissue dzs that can be ANA + | RA, scleroderma, Sjorgens, polymyositis and dermatomyositis
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| 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
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| 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,
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| tx SLE | NSAIDs, local or systemic steroids +/- hydroxychloroquine
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| 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
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| 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)
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| 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
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| what's the MC presenting sympt of scleroderma | Reynauds
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| what dz is commonly found w Scleroderma | Sjorgens (20%)
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| key Abs for scleroderma | CREST or limited=anti-centromere; diffuse=anti-Scl70 (or topoisomerase?)
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| CREST stands for | Calcinosis digits, raynauds, esophageal, sclerodactyly, telangiectasia (digits, nails)
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| 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
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| what need to monitor in scleroderma | esophageal motility w Ba swallow and PFTs (MC cause of death is pulmon)
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| describe sympt unique to diffuse scleroderma | ILD, peripheral edema, polyarteritis and carpal tunnel, fatigue, mscle involvement, + visceral involvement of lungs, heart, GI, kidney
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| describe pathophys of Sjorgen | lymphocytes invade lacrimal and salivary glands
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| describe 2 types of Sjorgens | primary w/o another connective tissue dz, 2ry w RA, sclero, SLE, polymyositis
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| what Ab + in Sjorgens | in 50% Ro (SS-A) and La (SS-B) are + [not specific]; note: SS-A at risk for neonatal SLE
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| tx Sjorgens | pilocarpine and eye drops
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| what Ab + in mixed connective tissue dz; describe the dz | anti-UI-RNP; overlap of dzs but don't nec occur simultaneously
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| pts who present w RA | usu women 20-40
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| 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
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| key findings accompany RA | C1, C2 cervical instability, pl eff common, pericarditis, episcleritis, rheum nodules
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| kids w uveitis, inflamm arthritis, RF -…what dz? | RA (often RF isn't + and in pauciarticular)
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| 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
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| MC cause of arthritis | osteoarthritis
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| key findings of OA on radiography | joint space narrowing, osteophytes, sclerosis of subchondral bone, subchondral cysts; Bouchards=PIP and Heberdens DIP
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| name of genetic syndrome assoc w gout | Lesch Nyhan defic in hypoxanthine guanidine phosphoryibosyltrxse
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| joint aspirate of gout v pseudogout v RA | all will have WBC>5,000, gout has needle shaped - birefringent, pseudogout has wkly + rod/rhomboid
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| 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
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| tx acute gout | NSAIDs (indomethacin) and colchicine [not ASA makes worse, not acetaminophin bc doesn't have anti-inflamm prop]
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| SE of colchicine | GI (80%): N/V, abd cramps, severe diarrhea
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| 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
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| 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]
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| what type of crystals form in pseudo gout | calcium pyrophosphate
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| 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)
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| how is pseudogout treated | esstly same as gout
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| clinical findings common to polymyositis and dermatomyositis | symmetrical prox mscle wknss (trbl getting up from chair, climbing steps), myalgia (1/3), dysphagia (1/3)
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| what skin findings seen in dermatomyositis | heliotrope rash, V sign, shawl sign, Gotton's papules=papular, eryth scaly lesions over knuckles
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| once dermatomyositis is dx what do you need to look for | other malignancy
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| 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
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| how is mscl bx different bw polymyositis and dermatomyositis | polymyositis (and inclusion body)=endomysial inflamm and fibrosis; dermatomyositis=perivascular and perimysial
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| 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
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| who does polymyalgia rheumatica occur in, what else to look for? | more elderly women, 10% have temporal arteritis
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| what are the muscles most often affects in polymyalgia rheumatica? | neck, shoulder, pelvic girdle [also for myositis]
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| what is clinical dx for fibromylagia | 11 of 18 points >3mos
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| 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
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| name the seronegative spondylarthropathies | ankylosing spondylitis, reactive arthritis/Reiters, psoriatic arthritis
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| what are clinical findings of ankylosing spondylitis | key is low back pain (bilateral sacroilitis) w limited motion, can have constitutional +/- uveitis
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| 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
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| classic Reiters characteristics | arthritis, uveitis, urethritis after enteric or GU infxn +/- genital and oral ulcers like Behcets
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| name the large artery vasculitis | Takayasu and temporal
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| name the med vessel arteritis (5) | PAN, Kawasaki, Wegeners, Churg Strauss, microscopic polyangitis
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| name the small arteritis (3) | HSP, hypersensitivity vasculitis, Behcets
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| 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
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| 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
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| 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
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| 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)
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| 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
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| common present of Behcets | young male 20s w painful oral and genital ulcers +/- uveitis, arthritis, eryth nodosum (but no prior infxn unlike Reiters)
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| dx, tx Behcets | need bx, steroids
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| 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
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| auto Ab: ANA | SLE (but not specific)
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| auto Ab: DNA | specific SLE
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| auto Ab: Smith | specific SLE
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| auto Ab: His | Rx induced Lupus
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| auto Ab: c-ANCA | Wegeners
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| auto Ab: p-ANCA | Churg Strauss, microscopic polyangitis
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| auto Ab: microsomal | Hashimotos thyroiditis
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| auto Ab: centromere | CREST scleroderma
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| auto Ab: Scl 70 | diffuse scleroderma
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| auto Ab: mitochondrial | primary biliary cirrhosis (scleroderma)
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| auto Ab: GBM | Goodpasture (more specific Col IV)
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| auto Ab: IgG | rheum arthrit
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| auto Ab: gliaden | celia sprue (allergic to gluten)
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| which Ab specific for Lupus | DNA and smith (His for Rx-induced Lupus)
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| HLAB27 assoc w | PAIR psoriasis, ankyl spond, inflamm bowel, Reiters
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| name seroneg arthritis | Reiters, ankyl spondyl, psioriatic arth (no rheum factor (anti IgG); all assoc HLA B27)
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| describe Reiters symptoms, cause | can't see, pee, climb tree (conjunctivitis/uveitis, urethritis, arthritis), usu s/p Gi or chlamydia
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| CREST | calcinosis, rayneud, esophag, sclerodactyly, telangiectesia
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| 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)
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| 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
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| ACID pneu for hypersensit | I A=anaphylactic, II C=cytotoxic, III I=immune complex, IV D=delayed
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| type I hypersensitivity incl | anaphylaxis, allergic rhinitis
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| type II hypersensitivity incl | hemolytic anemia, ITP, erythro blast fetalis, MG, Rheum F, Goodpasture, Graves, Bullous pemph
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| type III hypersensitivity incl | post-strep glomerulo, polyarteritis nodosum, RA, SLE
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| type IV hypersensitivity incl | MS, Guillan Barre, granulomas, PPD, contact dermatitis, DM type I
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| difft cholangitis and cholecystitis | cholangitis=bile duct, cholecystitis=gall bladder
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| what hypersensit type is SLE? | III
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| what hypersensit type is Hashimoto? | IV
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| what hypersensit type is Graves? | II
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| what hypersensit type is MS? MG? | MS=IV, MG=II
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| what hypersensit type is polyarteritis nodosa? | III
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| what hypersensit type is hyperacute transfusion? | II
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| what hypersensit type is hemo anemia? | II
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| what hypersensit type is ITP? | II
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| describe MS symptoms | SIN scan speech, intention tremor/INO/incontinence, nystagmus; sudden vision loss, paresthesia, periventricular plaques
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| describe MG symptoms | ptosis, diplopia, general PROX muscle weakness; symptoms get worse w muscle use
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| tx MS | beta interferon or immunosuppress
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| MG mech | auto Ab ag Ach R
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| dx, tx MG | dx: edrophonium, tx: pyridostigmine or neostigmine (AChE inhibitor)
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| 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
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