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random > malaise and pain clin- mcgowan > Flashcards

malaise and pain clin- mcgowan Flashcards

1
Q

homogenous ANCA is most associated with

A

drug induced SLE

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2
Q

specked ANCA is what antigens, and is mostly associated with what

A

UIRNP, SM +SSa (RO) SSb(la)

sjogren

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3
Q

anticentromere ANCA is mostly associated with what

A

CREST

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4
Q

nucleolar ANCA is what Ags and s mostly associated with what

A

antiRNA Abs (fibrillarin, RNA polymerase I+III, Th, PM-Scl, RNA helicase

DcSS

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5
Q

(+) serology with SLE

what virus can be associated with SLE

1/3 of SLE pts have what other autoimmune ds, diagnostic test?

what type of hypersensitivity is SLE

A

dsDNA, Sm, decreased C3+C4

EBV

antiphosholipid Ab syndrome (anticardiolipin, lupus anticoagulant, abn DRVVT)

type III hypersensitivity (Ag-Ab complexes–> necrotizing vasculitis)

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6
Q

what heme, cardiopulm, and neuro signs are associated with SLE

A

anemia, neutropenia, thrombocytopenia, thrombosis

pleural effusions, pericarditis,
atherosclerosis

seizures and psychosis

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7
Q

trx of SLE

SLE has increased risk of what complications

most likely cause of mortality

A

trx: nsaids, hydroxychloroquine, avoid sun, get vaccines regulary!!!

need preventative CA screens for risk of lymphoma, lung/cervical CA

-athersclerosis accelerated –> mortality with increased risk of MI, thrombosis
cardiorenal mortality associated in early disease

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8
Q

serology of drug induced lupus-like syndrome

A

+ANA, 95% antihistone Ab

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9
Q

neonatal lupus is seen in what population

sx?

A

babies born to moms w anti-Ro SSa or antiLA SSb

transient rashes, thrombocytopenia, hemolytic anemia and arthritis
permanent complete heart block possible

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10
Q

what is the hallmark of discoid lupus

A

(@ head)

well defined inflammatory plaques that evolve into atrophic, disfiguring scars

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11
Q

scleroderma

hallmark?

dcSSc vs lcSSc vs localized

A

halmark: thickened+hardened skin w fibrosis of the skin and visceral organs

dcSSc= diffuse= antiRNApol I+III, anti-Sc70**
–progressive UE/LE+trunk with early and progressive organ involvement
~interstitial lung ds, renal crisis

lcSSc= limited= anti-centeromere
–face/neck, distal UE/LE, raynauds=1st sx
CREST: calcinosis cutis, raynauds, esophageal dysmotility, sclerodactyly, telangiectasia
~digital ischemia, prog PULM HTN

localized: benign skin ds in children,
~morphea (pathces) that may coalesce

**anti-Sc70= topoisomerase I Ab

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12
Q

primary cause of morbidity and mortality in scleroderma

clin presentation of renal crisis in dcSSc

GI sx associated scleroderma

A

pulmonary ds (ILD in dcSSc, pulm HTN in lcSSC)

renal crisis= medical emergenc= abrupt onset MALIGNAN HTN, hemolytic anemia, progressive insuff
often inducible by high dose corticosteroids

fat, protein, vitB12, and Vit D deficiency
strictures+ Barrett’s esophagus (inc risk adenocarcinoma)
chronic diarrhea: PBC/cirrhosis associated with antimitochondrial Abs

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13
Q

trx of scleroderma

A

CCBs for raynauds

ACE-I for cardio/renal ds

anti-reflux meds

glucocorticoisd for myositis/pericarditis

cyclophosphamide for lung function and survival

PDE-5 Inhib : for PAH

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14
Q

sjogren

  • serology
  • inc risk of infection w what ? what kind of CA
  • clin test for keratoconjunctivits sicca
  • hallmark biopsy
  • trx : what drugs can you NOT use
A

(+) ANA, RF, anti-SSA(Ro) anti-SSB (La)

oral candida and dental caries : MALT lymphoma

schirmer test: measure quantity of tears with paper tails in eyes

LIP biopsy= essential for dx= lymphoid foci and accessory salivary glands

-regular appts w dentist, and ophtalmologist, artificial tears, hydroxychloroquine,
NO USE atropinic drugs or decongestants

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15
Q

dermatomyositis

skin findings

serology

biopsy
associated CAs

A

Gottron’s patches- raised, violatious patches @ dorsal DIP, PIP, MCP
heliotrope rash= raccoon eyes
periungal edema= V neck erythema= shawl sign

serology= +anti-Jo, anti-Mi2, anti-MDA5, anti-PI55/PI40

biopsy= PERIfascicular atrophy

associated w : occult malignancy anywhere
ovarian CA (most) (CA-125)
also lung, pancreas, stomach, colorectal, NHL

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16
Q

ages for dermato vs poly vs inclusion body myositis

polymyosiis: biopsy and serology

incusion body sx, biopsy, and serology

A

D= 7015 and 30-69
poly=30-50
IB= >40-50

P:
biopsy= ENDOmysial inlammation
anti-Jo1(Ab)

IB:
wk finger flexion or quadriceps wknss
biopsy= rimmed vacuoles
serology= anti-CNIA Ab

17
Q

IgA vasculitis=

-vessels?
population
sx

A

SMALL vessels

kids

tetrad: palpable purpura, arthritis/arthralgia, abd pain, glomerulonephritis

18
Q

antiGBM / goodpasture

-vessels
dx?

A

small = glomerular capillaries and pulm capillaries

dx RBCS and crescent shaped cells in urine, anti_GBM Ab deposits in BM of glomerulus and alveoli

19
Q 
takayasu arteritis
-vessels
-population
hallmark sxs
dx
complication
A

aorta, subclavian or brachiocephalic (aka innominate??) branches

-<40 yo, F, asian teens+YA

sx= pulseless ds (xUE pulses)
renal A stenosis
retinopathy w COPPER WIRING infarction

dx= w MRI/CT angio
histo= granuloma w giant cells and chronic stage fibrosis

complication: high risk of aortic rupture, can also have ischemia of limb but rarer and

20
Q

behcet syndrome

vessel involvement
ssx
dx
complication

A

-variable vessels, large vessels are associated with aneurysms, venous involvement, associated w DVT

TRIAD sx: RECURRENT mouth ulcers + genital ulcers + uveitis
+ileocecal ulcers, pathergy (pustules at site o needle prick)

dx= HLA-B51

complication: pulmonary embolus

21
Q

polyarteritis nodosa

population
sx
dx

A

males w HBV

livedo reticularis (lace like purple skin changes), 
subQ nodules, ulcers and distal gangrene, 
80% mononeuritis multiplex ~ FOOT DROP

NO pulm sx

dx: confirm w biopsy or angiogram,
1. fibrinoid necrosis w/O granulomas
2. microaneurysms

22
Q

kawasaki ds

population
sx
cause of death
trx
complication
A

=asian kids, <5 yo
sx: FEVER, lymphadenopathy, widespread rash, STRAWBERRY TONGUE, periungal peeling of skin

death~ coronary- aneurysms/MI years later

trx: IVIG w/in 10 days of onset, high dose ASA

MI or coronary artery aneurysm

23
Q

granulomatous polyangitis= wegener’s

population
sx
hallmark histo
serology
respiratory tract changes
A

M, >40 yo
sx= hearing loss, orbital mass, ulcerative keratitis, scleritis/conjunctivitis

hallmark: granulomatous inflammation, necrotizing vasculitis, SEGMENTAL glomerulonephritis,
serology: cANCA (PR3)

resp tract: saddle nost, alveolar hemorrhage (cavitary lesions)

24
Q

churg strauss / eosinophilic granulomatous w polyangiitis

prodrome
hallmark
serology

A

prodrome:

  1. allergic asthma/rhinits
  2. eosinophilia-tissue infiltration (into lung/GI)
  3. vasculitis phase: systemic necrotizing, palpable purpura

hallmark: asthma + eosinophilic –> vascular granulomas

ANCA= 50% MPO

25
Q

buerger ds = thromboangiiis obliterans

population
sx progression
dx

A

M, <35 yo
distal–> proximal vessels
dx: angiography= corkscrew appearance

26
Q

what two vasculitities often co-exist in white
Fs >40-50 yo

what are the dif sx, biopsy and lab results

A

giant cell arteritis/temporal arteritis and
polymyalgia rheumatica

GCA/TA =
temporal A>facial A>ophthalmic +aortic arch
sx- HA, jaw claudication, painless temporary loss of vision (amaurosis fugax), diplopia
tests= hla-dr4, inc ESR, gold standard is temporal A biopsy w segmental granulomatous vasc w multinucleated giant cells
start corticosteroids before even biopsy

PR:
sx= severe and symmetric morning stiffness, proximal (shoulder, neck, pelvic girdles)
tests: M biopsy shows no inflammation, M enzymes are normal, EMG is normal

27
Q

raynauds primary vs secondary
age
cause/associations
vasc involvement

A

primary: F 15-30 yo,
response to cold or emotion
normal vessel changes rom vasoconstriction–>ischemia–>reperfusion

secondary= >30 yo
unilat, thoracic outlet obstruction, carpal tunnels
vessels: distorted w wide and irregular loops, dilated lumen and vascular dropout

28
Q

skin lesions of discoid lupus vs morphea in localized scleroderma

A

discoid lupus: indurated scaly plaques

localized scleroderma= morphea (typical in kids)

29
Q

CCP Ab is associated with

A

RA

30
Q

describe endocarditis seen in Antiphospholipid Ab syndrome

A

libman sacks endocarditis
=sterile nodules, warty, mitral >aortic

M regurge= holosystolic murmur, blood return to the R atrium

31
Q

which serology finding is associated with SLE AND correlates with disease activity in patients

A

anti-ds DNA

32
Q

anti-mediastinal LAD is associated with what

A

CREST syndrome (limited scleroderma)

also sarcoidosis- inflammatory granulomas in multiple organs, especially lungs

33
Q

trx of IBM vs trx of PM/DM

A

PM/DM = steroids, hydroxychloroquine, IVIG, rituximab

IBM= supportive

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