DSA Malaise and Generalized Pain Flashcards by Roger Borelli

What is the significance of the ration of ANA?

titer of < 1:40 is normal/negative

higher the ration = more clinically significant

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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

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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 does a centromere nucleus staining pattern mean?

seen in CREST

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What does a nucleolar staining pattern mean?

Ab directed agains RNA

seen in SLE and diffuse systemic sclerosis

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What is libman-sacks endocarditis?

seen in SLE

vegetations are microthrombi

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Why do SLE pts have an increased risk of MI?

have accelerated atherosclerosis

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What is significant SLE serology?

+ ANA

+ anti-ds DNA (correlate w/ dz activity)

+ Smith

decreased C3 or C4

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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

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How do you treat antiphospholipid syndrome?

anticoagulation

continue indefinitely

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

SLE/ antiphospholipid syn retinopathy

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

+ ANA

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

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What drugs can cause a lupus-like syndrome?

hydralazine

Isoniazid

minocycline

TNF inhibitors

quinidine

chlorpromazine

methyldopa

procainamide

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How do sulfa drugs affect SLE?

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

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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

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What is the first line treatment for SLE?

avoid sun exposure

NSAIDs

corticosteroids

hydroxychloroquine

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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

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What is an MSK complication of SLE managed with corticosteroids?

avascular necrosis of bone

(hips and knees most commonly)

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What type of hypersensitivity is SLE?

type 3

(deposition of antigen-Ab complexes –> complement and leukocyte recruitment)

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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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How do you treat discoid lupus?

photoprotection + topical anti-inflammatory agents or systemic antimalarial drugs

(treat early to minimize scarring)

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What can discoid lupus be mistaken for?

tinea infection (ring worm)

psoriasis

morphea (limited scleroderma)

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What is the hallmark of scleroderma (SSc)?

thickening and hardening of the skin

micrangiopathy and fibrosis of skin and viscerlal organs

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Why do ppl with scleroderma have dry, itchy skin?

there is obliteration of eccrine sweat and sebaceous glands

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What are the 3 types of scleroderma?

diffuse (dcSSc) limited (lcSSc)/ CREST localized

What do you see in CREST syndrome/lcSSc

**C**utaneous calcinosis **R**aynaud's **E**sophageal dysmotility, GERD **S**clerodactaly (thickening and tightening of skin on fingers and hands) **T**telangiectasia \*Anti-centromere Abs \*vascular probs more common than w/ dcssc, get pulmonary A Htn

what are the two phases of Diffuse cutaneous systemic sclerosis?

inflammatory edematous phase --\> fibrotic phase

What are the internal organ involvements in dcSSc?

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

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

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

bronchoalveolar carcinoma

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 gastric pathology is specific to Scleroderma?

gastric antral vascular ectasia (GAVE) aka watermelon stomach

what msk pathologies can you see in scleroderma?

carpal tunnel syndrome tendon friction rubs fibrosis and adhesion of tendon sheaths

What endocrine dz is a complication of scleroderma?

hypothyroid from thyroid fibrosis

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

What is localized scleroderma?

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

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

sjogren syndrome

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 is the serology for sjogren's?

+ ana and RF high ESR hypergammaglobulinemia + anti SSA/Ro and anti SSB/La

What procedure is essential for diagnosis of sjogren's?

lip biopsy will see lymphoid foci in salivary glands

What drugs should ppl with sjogren's avoid?

atropinic drugs and decongestants

What do you see in dermatomyositis?

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

What labs do you see in dermatomyositis?

elevated CK and aldolase anti Jo and Anti Mi Abs

What is the feared association with dermatomyositis?

assoc with malignancy Ovarian most common

What do you see in polymositis?

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

What labs do you see in polymyositis?

elevated CK Anti- Jo 1 serology

What is the first line tx for dermatomyositis and polymyositis?

corticosteroids (do NOT use in inclusion body myositis)

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

How do you treat inclusion body myositis?

refractory to steroids, so tx is supportive

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

How do you treat IgA vasculitis?

supportive/steroids

What is goodpasture's syn?

anti-basiment membrane autoantibodies in kidney and lung capillaries

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

What is the triad of sx of behcet syndrome?

recurrent mouth ulcers genital ulcers eye inflammation

What is the HLA association to behcet syndrome?

HLA-B51

What vasculitis is assoc w/ HBV?

polyarteritis nodosa

What do you see on bx of polyarteritis nodosa?

fibrinoid necrosis, NO granulomas

What is kawasaki dz?

medium vessel vasculitis asian kid w/ fever, LAD, rash, strawberry tongue death from coronary involvement years later

How do you tx kawasaki dz?

IVIg and aspirin

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

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

What serology is positive in Wegener's

C-ANCA

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

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

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

MPO-ANCA

What is buerger dz/thromboangiitis obliterans

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

What msk dz is associated with giant cell arteritis?

polymyalgia rheumatica

How do you diagnose giant cell arteritis?

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

Which type of raynaud's is more severe?

secondary, get ischemia

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