Vasculitis II

Question 1

Henoch Schonlein Purpura

Answer 1

small vessel vasculitis that affects kids and YA (mostly). Causes PALPABLE PURPURA, ARTHRITIS, AND ABDOMINAL PAIN . Associated with mesangial proliferative nephritic or nephrotic kidney disease and fever.
elevated IgA levels with deposition in vessel walles. (this is a systemic form of Berger’s disease, which is limited to the kidney)

Question 2

Dx criteria for Henoch-Scholein purpura

Answer 2

2/4 features:

1. under 20 yo
2. palpable purpura
3. bowel angina (pain after meals or bowel ischemia with bloody diarrhea)
4. granulocytes in walls of arterioles or venules on biopsy

Question 3

cryoglobulinemia general features

Answer 3

immunoglobuins that undergo reversible precipitation in the cold
features related to cold precipitating proteins and to immune complex deposition:
cold precipitating protein sx: acral cyanosis, ulceration, and necrosis (acral = extremities) and Raynaud’s, ear necrosis
immune complex depostion: purpura, arthralgia, nephritis, neuropathy

Question 4

cryoglobulinemia type I

Answer 4

monoclonal IgM. associated with hematologic malignancy

Question 5

cryoglobulinemia type II

Answer 5

mixed IgM cryoglobulines (monoclonal) complexed with polyclonal IgG. Associated with HCV (important!) and infection, hematologic malignancies, systemic rheum diseases

Question 6

Type III cryoglobulinemia

Answer 6

polyclonal IgM with polyclonal IgG or IgA.

Question 7

Granulomatosis with polyangitis

Answer 7

aka Wegeners
destructive granulomas, esp in head and neck (larynx, upper airway, pharynx, nasal cartilage and sinuses). May cause episcleritis.
pulmonary infiltrates and cavitation
glomerulonephritis (pauci-immune crescenti)
c-ANCA positive
associated with silica inhalation and alpha1 antitrypsin deficiency.

Question 8

Churg-Strauss syndrome

Answer 8

asthma, eosinophilia >10%, migratory pulmonary opacities, paranasal sinus abnormality, granulomas with eosinophils in extravascular areas.
p-ANCA and IgE positive.
can cause cardiomyopathy and pericardial effusion, glomerulonephritis
3 stages

Question 9

microscopic polyangitis

Answer 9

small vessel vasculitis
may cause crescentic glomerulonephritis and pulmonary hemorrhage
pauci-immune- no immune complexes
lots of p-ANCA
GI involvement common

Question 10

olyarteritis nodosa vs. microscopic polyangitis: vessel size, renal invovlement, lung invovlement, ANCA

Answer 10

PAN: medium vessels, renal vascular nephropathy, no lung invovlement, ANCA negative
MPA: small vessels, glomerulonephritis, lungs involved in 1/3, ANCA posiitve
if you can see a problem via angiography, it is probably PAN not MPA

Question 11

jaw claudication, visual loss, palpable thickened temoporatl arteries associated vasculitis

Answer 11

giant cell arteritis

Question 12

absent radial pulse or unequal BP associated vasculitis

Answer 12

Takayasu’s or giant cell arteritis

Question 13

mononeuritis multiplex associated vasculitis

Answer 13

PAN, wegener’s (Granulomatosis with polyangitis) or churg struass. PAN is the most important cause here

Question 14

HTN due to renal artery involvement associated vasculitis

Answer 14

PAN or takayasu

Question 15

HTN due to glomerulonephritis associated vasculitis

Answer 15

wegner’s or MPA (microscopic polyangitis)

Question 16

asthma associated vasculitis

Answer 16

churg strauss

Question 17

testicular pain associated vasculitis

Answer 17

PAN

Question 18

bloody diarrhea and crampy abdominal pain associated vasculitis

Answer 18

henoch scholein purpura