Etiology and Outcome of Crescentic Glomerulonephritis From a Single Center in China: A 10-Year Review.
The disease spectrum of crescentic glomerulonephritis (GN) has been described in only a few previous studies, and detailed epidemiologic data from China are unavailable to date.
Case series.
528 patients with biopsy-proven crescentic GN in 2003 to 2013 from a single center.
Crescentic GN was classified into 3 types according to immunofluorescence findings: type I was defined as linear deposition of immunoglobulins along the glomerular basement membrane; type II, as glomerular deposition of immune complex; and type III, as pauci-immune deposition.
Demographic, clinical, and serologic characteristics.
Of 528 cases identified, 208 (39.4%) were men, with a mean age of 37.6±16.4 (SD) years at kidney biopsy. 61 (11.6%) patients had type I crescentic GN, 331 (62.7%) had type II (lupus nephritis, 34.3%; immunoglobulin A [IgA] nephropathy, 17.4%), and 136 (25.8%) had type III. Proportions of patients with acute kidney injury (AKI), acute kidney diseases and disorders without AKI, and chronic kidney disease were 86.9%, 0%, and 13.1% for type I; 42.0%, 19.6%, and 38.4% for type II; and 84.6%, 2.9%, and 12.5% for type III crescentic GN, respectively. Serum antineutrophil cytoplasmic antibodies were detected in 11 (18.0%) patients with type I, 15 (4.5%) with type II, and 117 (86.0%) with type III. Anti-glomerular basement membrane antibodies were found in 60 (98.4%) patients with type I, 3 (0.9%) with type II, and 5 (3.7%) with type III. 5-year cumulative renal survival rates for patients with types I, II, and III were 17.6%, 70.1%, and 44.3%, respectively.
Retrospective study, single-center experience.
Lupus nephritis may be the most common type of crescentic GN in China, followed by pauci-immune crescentic GN and IgA nephropathy. Almost half the patients presented with AKI, whereas 28.8% of cases showed chronic kidney disease. Clinical manifestations and outcomes varied according to crescentic GN type. The distinction between subtypes based on immunofluorescence and serologic findings has important implications for therapy and outcome.
Chen S
，Tang Z
，Xiang H
，Li X
，Chen H
，Zhang H
，Hu W
，Zeng C
，Liu Z
... -
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The evolution of crescentic nephritis and alveolar haemorrhage following induction of autoimmunity to glomerular basement membrane in an experimental model of Goodpasture's disease.
Goodpasture's, or anti-glomerular basement membrane (GBM), disease presents with rapidly progressive glomerulonephritis and lung haemorrhage, and is caused by autoimmunity to the NC1 domain of the alpha3 chain of type IV collagen (alpha3(IV)NC1). This study examines the development of crescentic nephritis and alveolar haemorrhage in a model of Goodpasture's disease, experimental autoimmune glomerulonephritis (EAG), induced in WKY rats by immunization with rat GBM in adjuvant. An increase in circulating anti-GBM antibodies and albuminuria was observed by week 2, which increased further by weeks 3 and 4, while a decrease in creatinine clearance was observed by week 2, which decreased further by weeks 3 and 4. The kidneys of animals with EAG showed linear deposits of IgG on the GBM and a transient glomerular infiltration by CD4+ T cells at week 2. By week 3 there were large deposits of fibrin in Bowman's space, and glomerular infiltration by CD8+ T cells and macrophages, accompanied by focal necrotizing glomerulonephritis with crescent formation. Ultrastructural studies showed glomerular endothelial cell swelling and epithelial cell foot process effacement at week 2. As the lesion progressed, capillary loops became occluded and the mesangium became expanded by mononuclear cells. By week 3 there was detachment of the endothelium from the GBM, and accumulation of fibrin beneath the disrupted endothelial cells and in Bowman's space. Occasional breaks were observed in the continuity of the basement membrane, and cytoplasmic projections from infiltrating mononuclear cells could be seen crossing the capillary wall between the lumen and the crescent. The lungs of animals with EAG showed patchy binding of IgG to the alveolar basement membrane (ABM) at week 2, and infiltration of the interstitium by CD8+ T cells and macrophages by weeks 3 and 4, accompanied by both interstitial and alveolar haemorrhage. Ultrastructural studies showed focal mononuclear cell infiltrates in alveolar walls at week 2. Occasional breaks were observed in the basement membrane and adjacent endothelium by weeks 3 and 4, together with accumulation of surfactant and erythrocytes within the alveolar spaces. This study defines for the first time the relationship between the immunological and pathological events during the evolution of EAG, and provides the basis for further work on the pathogenesis of Goodpasture's disease.
Reynolds J
，Moss J
，Duda MA
，Smith J
，Karkar AM
，Macherla V
，Shore I
，Evans DJ
，Woodrow DF
，Pusey CD
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《JOURNAL OF PATHOLOGY》
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