Clinical features and outcomes of anti-glomerular basement membrane disease in older patients.

Anti-glomerular basement membrane (GBM) disease is being recognized increasingly in older patients. Disease presentation and outcomes of these patients are unclear. Case series. 221 consecutive Chinese patients with anti-GBM disease diagnosed in 1998-2008 in our tertiary referral center. Anti-GBM disease was defined as positive anti-GBM antibodies in circulation and/or linear immunoglobulin G deposition along the GBM on kidney biopsy. Older age, defined as 65 years or older, and antineutrophil cytoplasmic antibody, detected using immunofluorescence and enzyme-linked immunosorbent assay, at presentation. Clinical features, kidney pathologic characteristics, end-stage renal disease (ESRD), and mortality. Multivariate Cox proportional hazard models were used to assess the contribution of age, sex, clinical measures, and treatments to ESRD and mortality. 50 of 221 (22.6%) patients were 65 years or older. Older patients had a male predominance (male/female ratio, 1.9:1). They had a higher proportion of positive antineutrophil cytoplasmic antibody results (46.0% vs 14.6%; P < 0.001), lower prevalence of hemoptysis (26.0% vs 46.2%; P = 0.01), lower urine protein excretion (1.4 ± 1.0 vs 3.9 ± 3.3 g/d; P = 0.001), and higher estimated glomerular filtration rate (eGFR) at presentation (8.4 vs 5.1 mL/min/1.73 m(2); P = 0.007) compared with younger patients. During follow-up, 30 of 37 (81.1%) and 21 of 37 (56.8%) patients developed ESRD and died in the older group compared with 115 of 139 (82.7%) and 35 of 139 (25.2%) in the younger group (P = 0.1 and P = 0.001, respectively). For older patients, multivariate Cox regression analysis showed that higher initial eGFR was an independent predictor for both ESRD (HR, 0.86; 95% CI, 0.78-0.96; P = 0.005) and death (HR, 0.79; 95% CI, 0.66-0.94; P = 0.008). Not all patients underwent kidney biopsy, especially those with very old age or ESRD at presentation. Older patients with anti-GBM disease had milder kidney damage and less pulmonary involvement. Outcomes were predicted by initial eGFR. Thus, early diagnosis was crucial to improve outcomes.

Cui Z ，Zhao J ，Jia XY ，Zhu SN ，Zhao MH ... -  《-》

Characteristics and prognosis of Chinese patients with anti-glomerular basement membrane disease.

Patients with anti-glomerular basement membrane (GBM) disease were predominantly reported in Caucasian population and reports from Chinese were lacking. The general picture of Chinese patients with anti-GBM disease was still unclear. This study is to investigate the characteristics and prognosis of Chinese patients with anti-GBM disease. Data from 105 patients with anti-GBM disease diagnosed in our hospital, between 1997 and 2002, were analyzed retrospectively. All the 105 sera were screened by enzyme-linked immunosorbent assay (ELISA) using highly purified bovine alpha(IV)NC1 as solid phase ligands. Clinical and pathological data of 69 patients with complete clinical remission (n = 5), partial remission (n = 10), and treatment failure (n = 54) were compared and the prognostic factors were evaluated. Patients increased chronologically and three quarters of the 105 patients were diagnosed in the last 3 years. Most of the patients were between 20 and 29 years (n = 31) and a smaller second peak was found in patients over 60 years. 25/105 (24%) were also ANCA-positive. Patients with both anti-GBM antibodies and ANCA positive were elder (50 +/- 19 vs. 34 +/- 15 years, p < 0.01) and female predominant (15/25 vs. 16/80, p < 0.05). 56/97 (58%) patients presented as Goodpasture syndrome, 40/97 (41%) patients presented as rapidly progressive glomerulonephritis and one patient had pulmonary hemorrhage only. The following factors predict poor prognosis: (1) serum creatinine more than 600 micromol/l on diagnosis (p < 0.01); (2) oliguria or anuria on diagnosis (p < 0.01); (3) a high percentage (>85%) of glomeruli had crescents (p < 0.01), and (4) renal involvement before pulmonary hemorrhage (p < 0.05). Patients with serum creatinine over 600 micromol/l on diagnosis had higher levels of anti-GBM antibodies (106 +/- 48% vs. 73 +/- 40%, p < 0.01). Intensive plasma exchange therapy predicts a better prognosis in the patients with serum creatinine less than 600 mumol/l (p < 0.05). Anti-GBM disease is not rare in China and behaves similarly to elsewhere. Early diagnosis and intensive plasmapheresis might be the most promising approaches to improve the outcome.

Cui Z ，Zhao MH ，Xin G ，Wang HY ... -  《nephron clinical practice》

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 ... -  《-》

Clinical outcome of patients with coexistent antineutrophil cytoplasmic antibodies and antibodies against glomerular basement membrane.

Lindic J ，Vizjak A ，Ferluga D ，Kovac D ，Ales A ，Kveder R ，Ponikvar R ，Bren A ... -  《-》

Anti-glomerular basement membrane disease in the elderly.

Kaplan AA 《-》