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 ... -  《-》

Crescentic Glomerulonephritis: an update on Pauci-immune and Anti-GBM diseases.

Kambham N 《-》

Glomerular capillary and endothelial cell injury is associated with the formation of necrotizing and crescentic lesions in crescentic glomerulonephritis.

Fujita E ，Nagahama K ，Shimizu A ，Aoki M ，Higo S ，Yasuda F ，Mii A ，Fukui M ，Kaneko T ，Tsuruoka S ... -  《-》

Membranous nephropathy with crescents: a series of 19 cases.

Membranous nephropathy (MN) with crescents is rare and, in the absence of lupus, usually is associated with anti-glomerular basement membrane (anti-GBM) nephritis or antineutrophil cytoplasmic antibody (ANCA)-positive glomerulonephritis. Only rare cases of crescentic MN without ANCA or anti-GBM have been reported. Case series. 19 patients with ANCA- and anti-GBM-negative crescentic MN and no clinical evidence of systemic lupus. Clinical features, kidney biopsy findings, laboratory results, treatment, and follow-up of patients with crescentic MN. Mean age was 55 (range, 5-86) years. All patients presented with proteinuria (mean protein excretion, 11.5 [range, 3.3-29] g/d) and nearly all had hematuria; 16 of 19 (84%) patients had decreased estimated glomerular filtration rates (eGFRs; mean serum creatinine, 2.9 [range, 0.4-10] mg/dL; mean eGFR, 39.7 [range, 4 to >100] mL/min/1.73 m2). Glomeruli showed on average 25% (range, 2%-73%) involvement by crescents. All showed a membranous pattern; 7 showed mesangial and 2 showed segmental endocapillary proliferation. By immunofluorescence, all cases showed granular subepithelial immunoglobulin G (IgG) and κ and λ light chains, and all but one showed C3; 5 showed C1q or IgA. Electron microscopy revealed stages I-III MN; 38% of cases were M-type phospholipase A2 receptor (PLA2R) associated, indicating that at least some were primary MN. Follow-up clinical data were available for all patients (mean, 22 [range, 1.5-138] months). 14 patients received immunosuppressive therapy, and 2, only angiotensin-converting enzyme inhibitor/angiotensin receptor blocker therapy. 4 patients (21%) progressed to end-stage renal disease, at 0-9 months postbiopsy. Mean serum creatinine level of those without end-stage renal disease at follow-up was 1.7 (range, 0.5-4.1) mg/dL; mean eGFR was 53.3 (range, 16-103) mL/min/1.73 m2. 67% of patients had proteinuria with protein excretion≥1 (mean, 3.2) g/d at follow-up. Retrospective study. Crescentic MN is a rare variant of MN that usually presents with heavy proteinuria, hematuria, and decline in GFR. The prognosis is variable and the disease may respond to therapy, but most patients develop a long-term decline in GFR.

Rodriguez EF ，Nasr SH ，Larsen CP ，Sethi S ，Fidler ME ，Cornell LD ... -  《-》

ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits.

Haas M ，Jafri J ，Bartosh SM ，Karp SL ，Adler SG ，Meehan SM ... -  《-》