malaise and pain clin- mcgowan Flashcards
homogenous ANCA is most associated with
drug induced SLE
specked ANCA is what antigens, and is mostly associated with what
UIRNP, SM +SSa (RO) SSb(la)
sjogren
anticentromere ANCA is mostly associated with what
CREST
nucleolar ANCA is what Ags and s mostly associated with what
antiRNA Abs (fibrillarin, RNA polymerase I+III, Th, PM-Scl, RNA helicase
DcSS
(+) 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
dsDNA, Sm, decreased C3+C4
EBV
antiphosholipid Ab syndrome (anticardiolipin, lupus anticoagulant, abn DRVVT)
type III hypersensitivity (Ag-Ab complexes–> necrotizing vasculitis)
what heme, cardiopulm, and neuro signs are associated with SLE
anemia, neutropenia, thrombocytopenia, thrombosis
pleural effusions, pericarditis,
atherosclerosis
seizures and psychosis
trx of SLE
SLE has increased risk of what complications
most likely cause of mortality
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
serology of drug induced lupus-like syndrome
+ANA, 95% antihistone Ab
neonatal lupus is seen in what population
sx?
babies born to moms w anti-Ro SSa or antiLA SSb
transient rashes, thrombocytopenia, hemolytic anemia and arthritis
permanent complete heart block possible
what is the hallmark of discoid lupus
(@ head)
well defined inflammatory plaques that evolve into atrophic, disfiguring scars
scleroderma
hallmark?
dcSSc vs lcSSc vs localized
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
primary cause of morbidity and mortality in scleroderma
clin presentation of renal crisis in dcSSc
GI sx associated scleroderma
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
trx of scleroderma
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
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
(+) 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
dermatomyositis
skin findings
serology
biopsy
associated CAs
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
ages for dermato vs poly vs inclusion body myositis
polymyosiis: biopsy and serology
incusion body sx, biopsy, and serology
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
IgA vasculitis=
-vessels?
population
sx
SMALL vessels
kids
tetrad: palpable purpura, arthritis/arthralgia, abd pain, glomerulonephritis
antiGBM / goodpasture
-vessels
dx?
small = glomerular capillaries and pulm capillaries
dx RBCS and crescent shaped cells in urine, anti_GBM Ab deposits in BM of glomerulus and alveoli
takayasu arteritis -vessels -population hallmark sxs dx complication
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
behcet syndrome
vessel involvement
ssx
dx
complication
-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
polyarteritis nodosa
population
sx
dx
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
kawasaki ds
population sx cause of death trx complication
=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
granulomatous polyangitis= wegener’s
population sx hallmark histo serology respiratory tract changes
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)
churg strauss / eosinophilic granulomatous w polyangiitis
prodrome
hallmark
serology
prodrome:
- allergic asthma/rhinits
- eosinophilia-tissue infiltration (into lung/GI)
- vasculitis phase: systemic necrotizing, palpable purpura
hallmark: asthma + eosinophilic –> vascular granulomas
ANCA= 50% MPO
buerger ds = thromboangiiis obliterans
population
sx progression
dx
M, <35 yo
distal–> proximal vessels
dx: angiography= corkscrew appearance
what two vasculitities often co-exist in white
Fs >40-50 yo
what are the dif sx, biopsy and lab results
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
raynauds primary vs secondary
age
cause/associations
vasc involvement
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
skin lesions of discoid lupus vs morphea in localized scleroderma
discoid lupus: indurated scaly plaques
localized scleroderma= morphea (typical in kids)
CCP Ab is associated with
RA
describe endocarditis seen in Antiphospholipid Ab syndrome
libman sacks endocarditis
=sterile nodules, warty, mitral >aortic
M regurge= holosystolic murmur, blood return to the R atrium
which serology finding is associated with SLE AND correlates with disease activity in patients
anti-ds DNA
anti-mediastinal LAD is associated with what
CREST syndrome (limited scleroderma)
also sarcoidosis- inflammatory granulomas in multiple organs, especially lungs
trx of IBM vs trx of PM/DM
PM/DM = steroids, hydroxychloroquine, IVIG, rituximab
IBM= supportive
