Kidney nontumor
Other primary glomerular diseases
Rapidly progressive (crescentic) glomerulonephritis TYPE 1

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 20 November 2018, last major update February 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

Related topic: Rapidly progressive (crescentic) glomerulonephritis
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Cite this page: Sangle, N. Rapidly progressive (crescentic) glomerulonephritis TYPE 1. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyrpgntype1.html. Accessed December 10th, 2019.
Definition / general
TYPE 1: Anti-glomerular basement membrane antibody crescentic glomerulonephritis (15%)

  • Goodpasture’s syndrome: antibody to alpha 3 chain of type 4 collagen (part of non-collagenous domain) in lung alveolar basement membrane; associated with smoking and HLA-DRB1
  • Usually young adult males with pulmonary involvement or age 50+ women limited to kidney
  • Pulmonary hemorrhage present, treated with plasmapheresis, steroids and cytotoxic agents
  • Most cases of Goodpasture’s are associated with rapidly progressive glomerulonephritis; but only 50% have both renal and pulmonary syndromes
  • Laboratory: 1/3 have circulating antineutrophil cytoplasmic antibodies (ANCA), especially ANCA specific for myeloperoxidase
Case reports
Microscopic (histologic) description
  • Hypercellular glomeruli, crescents in > 50% glomeruli at time of biopsy, variable neutrophils, no / rare intracapillary cell proliferation and earliest lesion is focal segmental fibrinoid necrosis
Microscopic (histologic) images

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Diffuse linear staining for IgG

Immunofluorescence description
  • Diffuse linear staining of glomerular basement membrane, IgG > IgM, also C3 and focal fibrin in capillary loops, IgG should be at least 3+ or 4+ and no staining in mesangium
Electron microscopy description
  • No deposits; fibrin at glomerular basement membrane breaks
Differential diagnosis
    Based on clinical presentation:
  • Microscopic polyarteritis
  • Granulomatosis with polyangiitis (Wegener's)
  • Similar immunofluorescent findings in diabetic nephropathy
  • SLE
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