Incidentally Diagnosed Alport Syndrome in a Patient with Drug-Induced Vasculitis

Box 1

Key Teaching Points.

Immunologic features that characterize Drug Induced ANCA Vasculitis (DIAV) include:

high titers of anti-MPO, dual positive anti-MPO and anti-PR3, anti-histone, and/or anti-

nuclear antibodies

Alport’s syndrome is diagnosed with the absence of alpha5 chain of type 4 collagen on

immunofluorescence and splitting / lamellation of the glomerular basement membrane

on electron micrography.

Even in the absence of proteinuria, renin-angiotensin-aldosterone system (RAAS)

inhibition can slow the progression of kidney disease in Alport’s syndrome.