Small vessel vasculitis

Question 1

Microscopic polyangiitis:

characteristics 3

Answer 1

1) Necrotizing vasculitis commonly involving lung, kidney, skin with
2) pauci-immune glomerulonephritis and
3) palpable purpura.

Question 2

Microscopic polyangiitis:

pathology 2

Answer 2

1) No granulomas
2) p-ANCA
Treat with cyclophosphamide and corticosteroid

Question 3

Wegener’s granulomatosis
(granulomatosis with polyangiitis):
characteristics 3

Answer 3

1) Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media and mastoiditis
2) Lower respiratory: hemoptysis, cough, dyspnea
3) Renal: hematuria, red cell cast

Question 4

Wegener’s granulomatosis
(granulomatosis with polyangiitis):
pathology triad and Ab, CXR

Answer 4

Triad:
focal necrotizing vasculitis
necrotizing granumolas in the lung/upper airway
necrotizing glomerulonephritis

c-ANCA

CXR: large nodular densities
Treat with cyclophosphamide and corticosteroid

Question 5

Churg-Strauss syndrome:

characteristics 4

Answer 5

1) Asthma,
2) sinusitis,
3) palpable purpura,
4) peripheral neuropathy (e.g. wrist/foot drop)

Can also involve heart, GI, kidneys
(pauci immune glomerulonephritis)

Question 6

Churg-Strauss syndrome:

pathology 4

Answer 6

1) Granulomatous,
2) necrotizing vasculitis with eosinophilia
3) p-ANCA,
4) elevated IgE level.

Question 7

Henoch-Schonlein purpura:

characteristics 3 + triad

Answer 7

1) Most common childhood systemic vasculitis
2) Often follows URI
3) Classic triad:
Skin (palpable purpura on buttocks, legs)
Arthralgia
GI (abd pain, melena, multiple lesions of same age)

Question 8

Henoch-Schonlein purpura:

pathology

Answer 8

1) Vasculitis 2/2 IgA complex deposition

2) Associated with IgA nephropathy.