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CARDIOvASCuLAR ``CARdIOvASCulAR—PATHOlOGY CARDIOvASCuLAR ``CARdIOvASCulAR—PATHOlOGY SECTION III 315

Vasculitides (continued)
EPIdEMIOlOGY/PRESENTATION NOTES
Small-vessel vasculitis (continued)
Eosinophilic Asthma, sinusitis, skin nodules or purpura, Granulomatous, necrotizing vasculitis with
granulomatosis with peripheral neuropathy (eg, wrist/foot drop). eosinophilia G.
polyangiitis (Churg- Can also involve heart, GI, kidneys (pauci- MPO-ANCA/p-ANCA,  IgE level.
Strauss) immune glomerulonephritis).
Granulomatosis Upper respiratory tract: perforation of nasal Triad:
with polyangiitis septum, chronic sinusitis, otitis media, ƒ Focal necrotizing vasculitis
(Wegener) mastoiditis. ƒ Necrotizing granulomas in lung and upper
Lower respiratory tract: hemoptysis, cough, airway
dyspnea. ƒ Necrotizing glomerulonephritis
Renal: hematuria, red cell casts. PR3-ANCA/c-ANCA H (anti-proteinase 3).
CXR: large nodular densities.
Treatment: cyclophosphamide, corticosteroids.
Immunoglobulin A Also called Henoch-Schönlein purpura. Vasculitis 2° to IgA immune complex
vasculitis Most common childhood systemic vasculitis. deposition.
Often follows URI. Associated with IgA nephropathy (Berger
Classic triad: disease).
ƒ Skin: palpable purpura on buttocks/legs I Treatment: supportive care, possibly
ƒ Arthralgias corticosteroids.
ƒ GI: abdominal pain (associated with
intussusception)
Microscopic Necrotizing vasculitis commonly involving No granulomas.
polyangiitis lung, kidneys, and skin with pauci-immune MPO-ANCA/p-ANCA J (anti-
glomerulonephritis and palpable purpura. myeloperoxidase).
Presentation similar to granulomatosis with Treatment: cyclophosphamide, corticosteroids.
polyangiitis but without nasopharyngeal
involvement.
Mixed Often due to viral infections, especially HCV. Cryoglobulins are immunoglobulins that
cryoglobulinemia Triad of palpable purpura, weakness, arthralgias. precipitate in the Cold.
May also have peripheral neuropathy and renal Vasculitis due to mixed IgG and IgM immune
disease (eg, glomerulonephritis). complex deposition.
A B LCC C D E
LM
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