B6.035 Prework 2: HSP

Question 1

2 major problems due to vasculitis

Answer 1

inflammatory cell infiltration causes thinning of vessel walls
-increased vascular permeability
-hemorrhage into the affected organ
narrowing of the affected vessel from intimal proliferation and thrombus formation
-causes ischemia of affected organs
-would see organ necrosis

Question 2

common manifestation sites of small-medium vessel vasculitis

Answer 2

skin, kidney, lungs

Question 3

pathology of immune complex mediated vasculitis

Answer 3

circulating immune complexes deposit in blood vessels

complement and Fc receptor mediated recruitment and activation of inflammatory cells

Question 4

characterize IgA vasculitis

Answer 4

small vessel vasculitis frequently preceded by viral resp infection
IgA dominant immune deposits (primarily IgA1)
involves skin, GI, joints, kidneys

Question 5

diagnostic criteria of HSP

Answer 5

palpable purpura not to due to thrombocytopenia +
one of:
-diffuse abdominal pain
-predominant IgA deposition
-acute arthritis
-renal involvement

Question 6

where are palpable purpura in HSP

Answer 6

lower extremities and buttocks

Question 7

GI involvement of HSP

Answer 7

nonspecific ab pain to ischemia and bleeding
melena or bloody stools
intussusception known complication

Question 8

how to identify kidney involvement with HSP

Answer 8

protein and/or blood on urinalysis

• 1/3 to 1/2 have kidney involvement
• glomerulonephritis indistinguishable from IgA nephropathy
• ESRD is rare
• HTN bad prognostic sign

Question 9

ttx of HSP

Answer 9

supportive care for arthritis and GI involvement
corticosteroids can help improve symptoms faster (long taper)
4-6 week resolution irrespective of ttx

Question 10

prognosis of HSP

Answer 10

1/3 of pts relapse

relapse usually more mild w only skin involvement

Question 11

goodpasture syndrome

Answer 11

small vessel only

characterized by presence of circulating and deposited antibodies directed against BM antigens

Question 12

epidemiology of goodpastures

Answer 12

bimodal age distribution in 3rd and 6th decades
80-90% of pts will present with RPGN
slightly more than 50% have concurrent lung hemorrhage

Question 13

diagnosis of goodpastures

Answer 13

anti-GBM circulating IgG

direct immunofluorescence for Ig is gold standard- linear deposits along GBM

Question 14

histo of goodpastures

Answer 14

crescent formation in 95% of patients

75% of glomeruli affected

Question 15

ttx for goodpastures

Answer 15

plasmapheresis to remove pathogenic Abs
corticosteroids and immunosuppressants to blunt production of Abs
longer ttx than HSP

Question 16

what is drug induced vasculitis

Answer 16

Ab mediated type 3 hypersensitivity - protein antigens from the drug bind antibody
disease caused by deposition of circulating Ab/Ag complexes in blood vessels
-activation of complement
-activation of inflamm cascade
-IgM, IgA, IgE or C3 deposits

Question 17

diagnosis of drug induced vasculitis

Answer 17

elevated ESR
125I-C1q binding assay shows circulating immune complexes
decreased C3 and C4 levels in serum

Question 18

ttx of drug induced vasculitis

Answer 18

stop offending agent
generally self resolves in 1-2 weeks
NSAIDs for fever/joint pain, antihistamines for pruritis