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Chapter 11 - Small Vessel Vasculitis and Selected Pediatric Vascular Diseases

from Section 4 - Vascular Diseases

Published online by Cambridge University Press:  10 August 2023

By
Thorsten Wiech ,
Silke R. Brix  and
Peter F. Zipfel
Edited by
Helen Liapis
Helen Liapis
Affiliation:
Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
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Summary

Small vessel vasculitides affecting the kidney destroy the integrity of the capillary wall and are grouped according to their appearance on immunofluorescence/immunohistochemistry. This chapter will concentrate on rapidly progressive glomerulonephritides of anti-glomerular basement membrane (GBM) and pauci-immune type, associated with anti-neutrophil cytoplasmic antibodies (ANCA). Both diseases usually cause a rapid decline in kidney function and show necrotizing, crescentic glomerulonephritis. Thrombotic microangiopathy (TMA) is a differential diagnosis of vasculitis. Childhood hypertension concludes this chapter.

Keywords

ANCA-associated vasculitis (AAV)anti-GBM diseasethrombotic microangiopathy (TMA)rapidly progressive glomerulonephritis (RPGN)granulomatosis with polyangiitis (GPA)microscopic polyangiitis (MPA)diffuse alveolar hemorrhage (DAH)pulmonary-renal syndrome (PRS)thrombotic thrombocytopenic purpura (TTP)hemolytic uremic syndrome (HUS)microangiopathic hemolytic anemia (MAHA)hypertension (HTN)renovascular HTN.
Type
Chapter
Information
Pediatric Nephropathology & Childhood Kidney Tumors , pp. 213 - 230
Publisher: Cambridge University Press
Print publication year: 2023

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