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

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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.

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Fig. 1

Modified from Rosales et al. [22]

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Authors and Affiliations

  1. Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy

    Vincenzo L’Imperio, Franco Ferrario & Fabio Pagni

  2. Department of Nephrology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy

    Elena Ajello & Federico Pieruzzi

  3. Department of Pathology, Hospital Sacco, Milan, Italy

    Manuela Nebuloni & Antonella Tosoni

Authors
  1. Vincenzo L’Imperio
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  2. Elena Ajello
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  3. Federico Pieruzzi
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  4. Manuela Nebuloni
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  5. Antonella Tosoni
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  6. Franco Ferrario
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  7. Fabio Pagni
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Correspondence to Fabio Pagni.

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The authors declare absence of any conflict of interests in the management of this paper.

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L’Imperio, V., Ajello, E., Pieruzzi, F. et al. Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis. J Nephrol 30, 503–509 (2017). https://doi.org/10.1007/s40620-017-0394-x

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  • DOI: https://doi.org/10.1007/s40620-017-0394-x

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