Antiglomerular basement membrane (GBM)-antibody-mediated disease with normal renal function

Ang, Celina; Savige, Judy; Dawborn, J. K.; Miach, P. J.; Heale, W. F.; Clarke, B F; Sinclair, Roger

doi:10.1093/ndt/13.4.935

Cited by 49 publications

(47 citation statements)

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“…Pulmonary hemorrhage probably leads to earlier presentation and diagnosis before anuric renal failure ensues. That may partly due to an increased awareness of anti-GBM disease manifesting as hemoptysis or pulmonary hemosiderosis prior to, or with minimal renal involvement [10]. However, in our series, there was no difference about the initial serum creatinine between patients with lung hemorrhage prior to and after nephritis (p > 0.05), and early diagnosis was not achieved on the patients with pulmonary hemorrhage prior to renal involvement (70 vs. 43 days).…”

Section: Discussioncontrasting

confidence: 54%

“…Since intensive therapy with a combination of immunosuppression and plasma exchange was introduced, recent investigations documented a better survival [4, 5, 6, 7, 8, 9]. Furthermore, 15–36% of patients with anti-GBM disease were reported with normal renal function in one study [10]. Anti-GBM disease has been reported predominantly in the Caucasian population, and reports from Chinese were lacking.…”

Section: Introductionmentioning

confidence: 99%

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Characteristics and Prognosis of Chinese Patients with Anti-Glomerular Basement Membrane Disease

Cui¹,

Zhao²,

Xu³

et al. 2005

Nephron Clin Pract

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Background: 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. Methods: 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 α(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. Results: 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 µmol/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 µmol/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 µmol/l (p < 0.05). Conclusions: 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.

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Section: Discussioncontrasting

confidence: 54%

Section: Introductionmentioning

confidence: 99%

Characteristics and Prognosis of Chinese Patients with Anti-Glomerular Basement Membrane Disease

Cui¹,

Zhao²,

Xu³

et al. 2005

Nephron Clin Pract

View full text Add to dashboard Cite

show abstract

“…Studies have reported 40-80% incidence of renal insufficiency in anti-GBM disease. [192021] In patients with rapidly progressive glomerulonephritis, Jennette et al . found raised serum creatinine at a presentation in most patients of anti-GBM nephritis (mean 9.7 ± 7.2 mg/dl).…”

Section: Discussionmentioning

confidence: 99%

Anti-glomerular basement membrane crescentic glomerulonephritis: A report from India and review of literature

Gupta¹,

Agrawal²,

Kaul³

et al. 2016

Indian J Nephrol

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Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune disease that most commonly presents as rapidly progressive glomerulonephritis with or without pulmonary involvement. It is characterized by the presence of antibodies directed to antigenic targets within glomerular and alveolar basement membranes. This study was performed to evaluate the clinicopathological features and outcome in anti-GBM crescentic glomerulonephritis (CrGN) at a tertiary care center in North India over a period of 9 years (January 2004 to December 2012). A diagnosis of anti-GBM CrGN was made in the presence of >50% crescents, linear deposits of IgG along GBM, and raised serum anti-GBM antibody titer. Of 215 cases of CrGN diagnosed during this period, 11 had anti-GBM CrGN. Anti-GBM CrGN was found at all ages but was most common in the third to fifth decade with no gender predilection (mean age 48 +/- 15 years, 13–67 years). Patients presented with a mean serum creatinine of 10.2 +/- 5.3 mg/dl and sub-nephrotic proteinuria. Pulmonary involvement was present in two patients. Myeloperoxidase-antineutrophil cytoplasmic antibody was positive in two (2/11) elderly patients. Follow-up was available in four patients for a range of 30-270 (mean 99.5 ± 114.5) days, two remained dialysis dependent while two died due to uremia and sepsis. Our findings show that anti-GBM disease is a rare cause of CrGN in India, accounting for only 5% of patients. It usually presents as a renal-limited disease and is associated with a poor renal outcome.

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“…27,28 A handful of cases of very mild kidney disease but strong linear GBM staining have been reported. 23,26,29,30 Interestingly, one of these cases was associated with an IgG4-predominant autoantibody specific for a quaternary-structure epitope of the NC1 hexamer of a3, a4, and a5 chains of collagen IV and thus could not be detected using tests based on monomeric Goodpasture antigen. 26 Our patient likely has a circulating monoclonal IgG1k anti-GBM antibody that we have not yet been able to detect.…”

Section: Discussionmentioning

confidence: 99%

Monoclonal IgG1κ Anti–Glomerular Basement Membrane Disease: A Case Report

Coley

Shirazian

D’Agati

2015

American Journal of Kidney Diseases

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Antiglomerular basement membrane (GBM)-antibody-mediated disease with normal renal function

Abstract: Patients with anti-GBM disease with normal renal function are not uncommon, and often have a good prognosis. There is less renal damage, possibly because of lower levels of circulating anti-GBM antibodies and less glomerular complement deposition.

Cited by 49 publications

References 0 publications

Characteristics and Prognosis of Chinese Patients with Anti-Glomerular Basement Membrane Disease

Characteristics and Prognosis of Chinese Patients with Anti-Glomerular Basement Membrane Disease

Anti-glomerular basement membrane crescentic glomerulonephritis: A report from India and review of literature

Monoclonal IgG1κ Anti–Glomerular Basement Membrane Disease: A Case Report

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