DSA Malaise and Generalized Pain Flashcards by Maren Osterholt

What does a nucleolar staining pattern mean?

Ab directed agains RNA

seen in SLE and diffuse systemic sclerosis

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Ppl with scleroderma are at an increased risk of what type of cancer?

bronchoalveolar carcinoma

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what are the two phases of Diffuse cutaneous systemic sclerosis?

inflammatory edematous phase –> fibrotic phase

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How do you diagnose giant cell arteritis?

temporal a bx (need 1 cm segment bc granulomas are segmental)

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What procedure is essential for diagnosis of sjogren’s?

lip biopsy

will see lymphoid foci in salivary glands

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What drugs should ppl with sjogren’s avoid?

atropinic drugs and decongestants

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What endocrine dz is a complication of scleroderma?

hypothyroid from thyroid fibrosis

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What is discoid lupus erythematosus?

variant of cutaneous lupus

can be seen alone or as a manifestation of SLE

most commonly on the head

inflammatory plaques that evolve into gross scars

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What is the HLA association to behcet syndrome?

HLA-B51

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What labs do you see in polymyositis?

elevated CK

Anti- Jo 1 serology

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What serology is positive in Wegener’s

C-ANCA

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What is the serology for sjogren’s?

+ ana and RF

high ESR

hypergammaglobulinemia

+ anti SSA/Ro and anti SSB/La

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What is takayasu arteritis?

large vessels

< 40 yrs, W>M

long smooth tapered stenosis

pulseless dz

can have renal A stenosis and HTN

aortic dilatations and aneurysms

granulomatous w/ some giant cells

tx: steroids

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What does a speckled nuclear staining pattern mean?

anti Sm, Ro and La, Wcl-70, Jo and Mi

seen in:

Sjogren’s

Mixed CT Dz

diffuse systemic sclerosis

SLE

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What is the first line tx for dermatomyositis and polymyositis?

corticosteroids (do NOT use in inclusion body myositis)

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what msk pathologies can you see in scleroderma?

carpal tunnel syndrome

tendon friction rubs

fibrosis and adhesion of tendon sheaths

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What is goodpasture’s syn?

anti-basiment membrane autoantibodies in kidney and lung capillaries

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What is the serology specific to diffuse and limited SSc?

diffuse: anti-Scl 70 aka anti DNA topoisomerase I; anti-RNA polymerase III
limited: anti-centromere

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What do you see in CREST syndrome/lcSSc

Cutaneous calcinosis

Raynaud’s

Esophageal dysmotility, GERD

Sclerodactaly (thickening and tightening of skin on fingers and hands)

Ttelangiectasia

*Anti-centromere Abs

*vascular probs more common than w/ dcssc, get pulmonary A Htn

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What labs do you see in dermatomyositis?

elevated CK and aldolase

anti Jo and Anti Mi Abs

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What does a centromere nucleus staining pattern mean?

seen in CREST

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What CT autoimmune disorder has a strong association with B cell non-hodgkin lymphoma (MALT lymphoma)?

sjogren syndrome

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What msk dz is associated with giant cell arteritis?

polymyalgia rheumatica

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How do you treat inclusion body myositis?

refractory to steroids, so tx is supportive

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What do you see in sjogren's?

sicca sx = dry mouth, eyes, etc keratoconjuctivitis sicca = foreign body sensation in eyes bc of low tear production

What are the 3 types of antiphospholipid antibodies?

type 1 - causes false-positive tests for syphilis type 2 - lupus anticoagulant type 3 - anti-cardiolipin antibodies, directed at Beta2GPI

What do you see on bx of polyarteritis nodosa?

fibrinoid necrosis, NO granulomas

What does a renal crisis in scleroderma look like?

abrupt onset of malignant htn, hemolytic anemia, and progressive renal insufficiency more common in diffuse SSc \*high dose steroids can induce a crisis

What is an MSK complication of SLE managed with corticosteroids?

avascular necrosis of bone | (hips and knees most commonly)

What is the significance of the ration of ANA?

titer of \< 1:40 is normal/negative higher the ration = more clinically significant

What does eosinophilic granulomatosis w/ plyangiitis/Churg-strauss look like?

asthma + eosinophilia --\> vasculitis w/ granulomas \*no nose involvement (vs wegener's) palpable purpura

How do you treat antiphospholipid syndrome?

anticoagulation continue indefinitely

What is significant SLE serology?

+ ANA + anti-ds DNA (correlate w/ dz activity) + Smith decreased C3 or C4

What is IgAV aka henoch-scholein purpura?

IgA depositis in SMALL vessels usually in kids tetrad: palpable purpura (no thrombocytopenia), arthralgia, abd pain, renal dz

What is buerger dz/thromboangiitis obliterans

smoker --\> vasculitis in fingers and then fingers turn black tx = Stop smoking

What do you see in polymositis?

proximal m weakness NO skin changes like DM endomysial inflamation on bx

What do you see in inclusion body myositis?

weakness in finger flexion or quads rimmed vacuoles on bx CK mild elevation or normal anti-cN1A Abs

What is the feared association with dermatomyositis?

assoc with malignancy Ovarian most common

What are the internal organ involvements in dcSSc?

renal crisis w/ hemolytic anemia on labs interstitial lung dz, PAH

How do you tx kawasaki dz?

IVIg and aspirin

What is the first line treatment for SLE?

avoid sun exposure NSAIDs corticosteroids hydroxychloroquine

What is neonatal lupus and what is its feared complication?

affects kids of mothers w/ anti Ro or La Abs can cause permanent complete heart block

How do you treat discoid lupus?

photoprotection + topical anti-inflammatory agents or systemic antimalarial drugs (treat early to minimize scarring)

What serology is sometimes positive in eosinophilic granulomatosis w/ polyangiitis?

MPO-ANCA

How do sulfa drugs affect SLE?

induce a lupus flair (doesn't cause lupus-like syndrome)

How do you treat IgA vasculitis?

supportive/steroids

What are the 3 types of scleroderma?

diffuse (dcSSc) limited (lcSSc)/ CREST localized

What is a homogeneous staining pattern indicative of?

(entire nucleus is diffusely stained) anti DNA, histone, or DNP drug-induced lupus sjogren's syndrome SLE

What vasculitis is assoc w/ HBV?

polyarteritis nodosa

Why do ppl with scleroderma have dry, itchy skin?

there is obliteration of eccrine sweat and sebaceous glands

What are the main causes of death early and late in SLE?

early: infections, kidney, or CNS dz Late: accelerated athersclerosis and MI, Thromboembolic events

How do you dx raynaud's?

nailfold capillaroscopy: normal in primary secondary = distorted w/ widened and irregular loops, dilated lumen and areas of vascular dropout

What do you see in dermatomyositis?

gottron's papules heliotrope rash periungual erythema shawl-shaped erythema perimysial and perivascular inflammation perifascicular atrophy

What type of hypersensitivity is SLE?

type 3 (deposition of antigen-Ab complexes --\> complement and leukocyte recruitment)

What is the triad of sx of behcet syndrome?

recurrent mouth ulcers genital ulcers eye inflammation

What is localized scleroderma?

benign skin condition most often in kids discreet areas of discolored skin induration histologically indistinguishable from SSc patches = morphea

What drugs can cause a lupus-like syndrome?

hydralazine Isoniazid minocycline TNF inhibitors quinidine chlorpromazine methyldopa procainamide

What gastric pathology is specific to Scleroderma?

gastric antral vascular ectasia (GAVE) aka watermelon stomach

What is kawasaki dz?

medium vessel vasculitis asian kid w/ fever, LAD, rash, strawberry tongue can die from coronary aneurysm years later

What does granulomatosis w/ polyangiitis/wegener's look like?

granulomatous inflammation and necrotizing vasculitis \*nasal involvement + eyes, kidneys, lungs, and skin

What are the main causes of death in the systemic scleroses (3)?

aspiration pneumonia bc of GERD diffuse = interstitial lung dz (dry cough, velcro crackles, dx by PFT) limited = Pulmonary A htn

Why do SLE pts have an increased risk of MI?

have accelerated atherosclerosis

What serology do you see in a lupus-like syndrome/drug-induced lupus?

+ ANA + anti-histone Abs (95%, diffuse staining pattern)

What can discoid lupus be mistaken for?

tinea infection (ring worm) psoriasis morphea (limited scleroderma)

Which type of raynaud's is more severe?

secondary, get ischemia

What is libman-sacks endocarditis?

seen in SLE vegetations are microthrombi

What autoimmune dz can cause cotton wool spots on retina exam?

SLE/ antiphospholipid syn retinopathy

What is the hallmark of scleroderma (SSc)?

thickening and hardening of the skin micrangiopathy and fibrosis of skin and viscerlal organs