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Clinical Features and Outcomes in Patients With Membranous Nephropathy and Crescent Formation
1From the Renal Division, Department of Medicine, Peking University First Hospital; Institute of Nephrology, Peking University; Key Laboratory of Renal Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing (JW, PZ, ZC, ZQ, Y-mZ, FW, XW, J-wW, GL, F-DZ, M-hZ); Renal Division (PZ), Department of Medicine, The First College of Clinical Medical Science, China Three Gorges University, Yichang; Department of Biostatistics (S-nZ), Peking University First Hospital; and Peking-Tsinghua Center for Life Sciences (M-hZ), Beijing, China.
Abstract:
Cases of membranous nephropathy (MN) with crescent formation, in the absence of lupus, hepatitis B virus infection, anti-glomerular basement membrane (GBM) nephritis, or antineutrophil cytoplasmic antibody (ANCA), are on record. Clinical presentation and treatment outcomes in these patients are unclear. All patients with biopsy-proven MN diagnosed between years 2008 and 2014 and followed up were enrolled retrospectively. Patients with ANCA, anti-GBM antibodies, lupus, hepatitis B virus infection, or malignance were excluded. Clinical features and outcomes were compared between MN patients with and without crescent. Out of 401 consecutive patients with idiopathic MN, 28 (6.9%) showed crescent formation in 4.9% (2.2%-16.7%) of glomeruli. Mean age of these patients was 50.1 ± 11.1 years, and they presented with heavy proteinuria (6.5 ± 4.8 g/24 h) and hematuria; 21.4% of these patients had declined estimated glomerular filtration rate (<60 mL/min/1.73 m2) on biopsy. Anti-phospholipase A2 receptor antibody was detectable in 79.7% of these patients. These clinical features were comparable to the MN patients without crescent (P > 0.05). Twelve (42.9%) patients received steroids plus immunosuppressive therapy similar to that in patients without crescent (41.3%). Fewer patients with crescents achieved remission (67.9% vs 86.7%, P = 0.029). Crescent formation was a risk factor for no response to the treatments (odds ratio [OR] = 3.1, P = 0.033). Higher percentage of crescents predicted more risk for no remission (OR = 1.2, P = 0.038). Patients with crescents presented more frequencies of abnormal serum creatinine during follow-up (10.7% vs 1.3%, P = 0.031). Crescent formation was also a risk factor for worse renal outcome (relative risk = 10.2, P = 0.046). MN patients with crescents showed unfavorable therapeutic response and tended to have worse renal outcomes. More aggressive treatments and renal protection might be considered to improve the outcomes.
Insights
Membranous nephropathy with crescent formation, a rare variant, presents similarly to typical cases but shows poorer treatment response. Crescent presence indicates a higher risk of treatment non-response and worse kidney outcomes, suggesting a need for aggressive management.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Idiopathic membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults.
- Crescent formation in MN, though uncommon, raises questions about its clinical impact and prognostic significance.
- Exclusion of secondary causes like lupus, viral infections, and anti-GBM disease is crucial for defining idiopathic MN with crescents.
Purpose of the Study:
- To investigate the clinical presentation, treatment outcomes, and renal prognosis of patients with idiopathic membranous nephropathy (MN) and crescent formation.
- To compare these features with MN patients lacking crescents.
- To identify crescent formation as a potential predictor of treatment response and renal outcomes.
Main Methods:
- Retrospective analysis of 401 patients with biopsy-proven idiopathic MN diagnosed between 2008-2014.
- Exclusion of patients with secondary causes of MN (lupus, HBV, anti-GBM, ANCA, malignancy).
- Comparison of clinical, serological, and renal outcomes between MN patients with and without crescent formation.
Main Results:
- 28 patients (6.9%) had MN with crescent formation, presenting with heavy proteinuria and hematuria.
- Anti-phospholipase A2 receptor antibodies were detected in 79.7% of crescentic MN patients.
- Patients with crescents had significantly lower remission rates (67.9% vs 86.7%) and higher rates of abnormal serum creatinine during follow-up.
- Crescent formation was an independent risk factor for treatment non-response (OR=3.1) and worse renal outcomes (RR=10.2).
Conclusions:
- Idiopathic membranous nephropathy with crescent formation shares clinical features with non-crescentic MN but demonstrates a poorer therapeutic response.
- Crescent presence is a significant predictor of unfavorable treatment outcomes and adverse renal prognosis in MN.
- Consideration of more aggressive treatment strategies and renal protection is warranted for MN patients with crescents.
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