Page 287 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 14

Systemic Vasculitis and the Kidney:
ANCA Vasculitis and
Glomerulonephritis

Elizabeth J. Brant, M.D.1, 2?, Julie G. McGregor, M.D.1, 2,
J. Charles Jennette, M.D.1, 3, Ronald J. Falk, M.D.1-3 and

William F. Pendergraft III, M.D., Ph.D.1, 2

1University of North Carolina (UNC) Kidney Center, Chapel Hill, NC, US
2Division of Nephrology and Hypertension, Department of Medicine,
UNC-Chapel Hill, Chapel Hill, NC, US
3Department of Pathology and Laboratory Medicine, UNC-Chapel Hill,
Chapel Hill, NC, US

Abstract

Systemic vasculitides affecting small- and medium-sized vessels are particularly
likely to involve the kidneys. These vasculitides include microscopic polyangiitis,
granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis, IgA
vasculitis, polyarteritis nodosa, and cryoglobulinemic vasculitis. The most common
culprits of kidney injury in adults are microscopic polyangiitis, granulomatosis with
polyangiitis, and eosinophilic granulomatosis with polyangiitis, which predominantly
affect small vessels and are typically associated with the presence of anti-neutrophil
cytoplasmic autoantibodies. For this reason, we focus here on anti-neutrophil cytoplasmic
autoantibody-associated vasculitides. Over the past three decades, tremendous progress
has been made through investigative efforts to understand etiologic, pathogenetic and
clinical underpinnings of these disorders. We highlight notable their findings that
represent key breakthroughs in the field and proceed to the clinical presentation,
diagnosis, and an up-to-date discussion of studies related to clinical management for
inducing and maintaining remission. Finally, we review key prognostic indicators of
renal outcomes and predictors of treatment resistance and relapse. Although these
diseases have been transformed from ones that were usually fatal to ones that are now

? Correspondence: Elizabeth J. Brant M.D., University of North Carolina (UNC) Kidney Center, Chapel Hill, NC
27599 USA. Email: [email protected]

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