DSA Malaise and Generalized Pain Flashcards

1
Q

What is the significance of the ration of ANA?

A

titer of < 1:40 is normal/negative

higher the ration = more clinically significant

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2
Q

What is a homogeneous staining pattern indicative of?

A

(entire nucleus is diffusely stained)

anti DNA, histone, or DNP

drug-induced lupus

sjogren’s syndrome

SLE

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3
Q

What does a speckled nuclear staining pattern mean?

A

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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4
Q

What does a centromere nucleus staining pattern mean?

A

seen in CREST

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5
Q

What does a nucleolar staining pattern mean?

A

Ab directed agains RNA

seen in SLE and diffuse systemic sclerosis

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6
Q

What is libman-sacks endocarditis?

A

seen in SLE

vegetations are microthrombi

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7
Q

Why do SLE pts have an increased risk of MI?

A

have accelerated atherosclerosis

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8
Q

What is significant SLE serology?

A

+ ANA

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

+ Smith

decreased C3 or C4

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9
Q

What are the 3 types of antiphospholipid antibodies?

A

type 1 - causes false-positive tests for syphilis

type 2 - lupus anticoagulant

type 3 - anti-cardiolipin antibodies, directed at Beta2GPI

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10
Q

How do you treat antiphospholipid syndrome?

A

anticoagulation

continue indefinitely

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11
Q

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

A

SLE/ antiphospholipid syn retinopathy

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12
Q

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

A

+ ANA

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

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13
Q

What drugs can cause a lupus-like syndrome?

A

hydralazine

Isoniazid

minocycline

TNF inhibitors

quinidine

chlorpromazine

methyldopa

procainamide

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14
Q

How do sulfa drugs affect SLE?

A

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

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15
Q

What is neonatal lupus and what is its feared complication?

A

affects kids of mothers w/ anti Ro or La Abs

can cause permanent complete heart block

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16
Q

What is the first line treatment for SLE?

A

avoid sun exposure

NSAIDs

corticosteroids

hydroxychloroquine

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17
Q

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

A

early: infections, kidney, or CNS dz

Late: accelerated athersclerosis and MI, Thromboembolic events

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18
Q

What is an MSK complication of SLE managed with corticosteroids?

A

avascular necrosis of bone

(hips and knees most commonly)

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19
Q

What type of hypersensitivity is SLE?

A

type 3

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

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20
Q

What is discoid lupus erythematosus?

A

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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21
Q

How do you treat discoid lupus?

A

photoprotection + topical anti-inflammatory agents or systemic antimalarial drugs

(treat early to minimize scarring)

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22
Q

What can discoid lupus be mistaken for?

A

tinea infection (ring worm)

psoriasis

morphea (limited scleroderma)

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23
Q

What is the hallmark of scleroderma (SSc)?

A

thickening and hardening of the skin

micrangiopathy and fibrosis of skin and viscerlal organs

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24
Q

Why do ppl with scleroderma have dry, itchy skin?

A

there is obliteration of eccrine sweat and sebaceous glands

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25
Q

What are the 3 types of scleroderma?

A

diffuse (dcSSc)

limited (lcSSc)/ CREST

localized

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26
Q

What do you see in CREST syndrome/lcSSc

A

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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27
Q

what are the two phases of Diffuse cutaneous systemic sclerosis?

A

inflammatory edematous phase –> fibrotic phase

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28
Q

What are the internal organ involvements in dcSSc?

A

renal crisis w/ hemolytic anemia on labs

interstitial lung dz, PAH

29
Q

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

A

aspiration pneumonia bc of GERD

diffuse = interstitial lung dz (dry cough, velcro crackles, dx by PFT)

limited = Pulmonary A htn

30
Q

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

A

bronchoalveolar carcinoma

31
Q

What does a renal crisis in scleroderma look like?

A

abrupt onset of malignant htn, hemolytic anemia, and progressive renal insufficiency

more common in diffuse SSc

*high dose steroids can induce a crisis

32
Q

What gastric pathology is specific to Scleroderma?

A

gastric antral vascular ectasia (GAVE)

aka watermelon stomach

33
Q

what msk pathologies can you see in scleroderma?

A

carpal tunnel syndrome

tendon friction rubs

fibrosis and adhesion of tendon sheaths

34
Q

What endocrine dz is a complication of scleroderma?

A

hypothyroid from thyroid fibrosis

35
Q

What is the serology specific to diffuse and limited SSc?

A

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

36
Q

What is localized scleroderma?

A

benign skin condition most often in kids

discreet areas of discolored skin induration

histologically indistinguishable from SSc

patches = morphea

37
Q

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

A

sjogren syndrome

38
Q

What do you see in sjogren’s?

A

sicca sx = dry mouth, eyes, etc

keratoconjuctivitis sicca = foreign body sensation in eyes bc of low tear production

39
Q

What is the serology for sjogren’s?

A

+ ana and RF

high ESR

hypergammaglobulinemia

+ anti SSA/Ro and anti SSB/La

40
Q

What procedure is essential for diagnosis of sjogren’s?

A

lip biopsy

will see lymphoid foci in salivary glands

41
Q

What drugs should ppl with sjogren’s avoid?

A

atropinic drugs and decongestants

42
Q

What do you see in dermatomyositis?

A

gottron’s papules

heliotrope rash

periungual erythema

shawl-shaped erythema

perimysial and perivascular inflammation

perifascicular atrophy

43
Q

What labs do you see in dermatomyositis?

A

elevated CK and aldolase

anti Jo and Anti Mi Abs

44
Q

What is the feared association with dermatomyositis?

A

assoc with malignancy

Ovarian most common

45
Q

What do you see in polymositis?

A

proximal m weakness

NO skin changes like DM

endomysial inflamation on bx

46
Q

What labs do you see in polymyositis?

A

elevated CK

Anti- Jo 1 serology

47
Q

What is the first line tx for dermatomyositis and polymyositis?

A

corticosteroids (do NOT use in inclusion body myositis)

48
Q

What do you see in inclusion body myositis?

A

weakness in finger flexion or quads

rimmed vacuoles on bx

CK mild elevation or normal

anti-cN1A Abs

49
Q

How do you treat inclusion body myositis?

A

refractory to steroids, so tx is supportive

50
Q

What is IgAV aka henoch-scholein purpura?

A

IgA depositis in SMALL vessels

usually in kids

tetrad: palpable purpura (no thrombocytopenia), arthralgia, abd pain, renal dz

51
Q

How do you treat IgA vasculitis?

A

supportive/steroids

52
Q

What is goodpasture’s syn?

A

anti-basiment membrane autoantibodies in kidney and lung capillaries

53
Q

What is takayasu arteritis?

A

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

54
Q

What is the triad of sx of behcet syndrome?

A

recurrent mouth ulcers

genital ulcers

eye inflammation

55
Q

What is the HLA association to behcet syndrome?

A

HLA-B51

56
Q

What vasculitis is assoc w/ HBV?

A

polyarteritis nodosa

57
Q

What do you see on bx of polyarteritis nodosa?

A

fibrinoid necrosis, NO granulomas

58
Q

What is kawasaki dz?

A

medium vessel vasculitis

asian kid w/ fever, LAD, rash, strawberry tongue

death from coronary involvement years later

59
Q

How do you tx kawasaki dz?

A

IVIg and aspirin

60
Q

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

A

granulomatous inflammation and necrotizing vasculitis

*nasal involvement + eyes, kidneys, lungs, and skin

61
Q

What serology is positive in Wegener’s

A

C-ANCA

62
Q

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

A

asthma + eosinophilia –> vasculitis w/ granulomas

*no nose involvement (vs wegener’s)

palpable purpura

63
Q

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

A

MPO-ANCA

64
Q

What is buerger dz/thromboangiitis obliterans

A

smoker –> vasculitis in fingers and then fingers turn black

tx = Stop smoking

65
Q

What msk dz is associated with giant cell arteritis?

A

polymyalgia rheumatica

66
Q

How do you diagnose giant cell arteritis?

A

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

67
Q

Which type of raynaud’s is more severe?

A

secondary, get ischemia

68
Q

How do you dx raynaud’s?

A

nailfold capillaroscopy:

normal in primary

secondary = distorted w/ widened and irregular loops, dilated lumen and areas of vascular dropout