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J Nephrol. 2017 Aug;30(4):503-509. doi: 10.1007/s40620-017-0394-x. Epub 2017 Apr 5.

Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis.

Author information

1
Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
2
Department of Nephrology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
3
Department of Pathology, Hospital Sacco, Milan, Italy.
4
Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy. Fabio.pagni@unimib.it.

Abstract

Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. In this review will be discussed the entire spectrum of renal anti-GBM related conditions, focusing the attention on the differences in terms of pathogenesis, diagnosis and therapy of these disparate entities.

KEYWORDS:

Anti-GBM disease; Crescentic glomerulonephritis; Nephropathology; Rapidly progressive glomerulonephritis

PMID:
28382508
DOI:
10.1007/s40620-017-0394-x
[Indexed for MEDLINE]

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