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Membranous Glomerulonephritis With Crescents
Aikaterini Nikolopoulou1, Isabel Huang-Doran2, Stephen P McAdoo1
1Centre for Inflammatory Disease, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK.
Introduction:
Membranous glomerulonephritis (MGN) is rarely associated with necrotizing and crescentic glomerulonephritis (NCGN).
Methods:
We report the clinical and pathologic findings in 15 patients with MGN and NCGN associated with anti-neutrophil cytoplasm antibodies (ANCAs), anti-glomerular basement membrane (GBM), or anti-phospholipase A2 receptor (PLA2R) antibodies.
Results:
The cohort consisted of 15 patients: 7 males and 8 females with a median age of 63 years (range: 18-79). In 12 of 15 patients, MGN and NCGN were diagnosed at the time of the biopsy, and in 3 cases, MGN predated the NCGN. ANCA was positive in 7 cases (6 MPO myeloperoxidase (MPO)-ANCA and 1 PR3-ANCA), anti-GBM antibodies were detected in 5 cases, and anti-PLA2R antibodies were found in 2 cases. One case was negative for all antibodies. Microscopic hematuria was present in all but one patient who was anuric, and median urinary protein-to-creatinine ratio was 819.5 mg/mmol (range: 88-5600). Pathologic evaluation revealed MGN and NCGN with crescents involving 28% of glomeruli (median; range: 5%-100%). Follow-up was available for all 15 patients; all were treated with steroids; 10 with cyclophosphamide, and 6 also received rituximab. At a median follow-up of 72 months, 9 had stabilization or improvement of renal function, 6 had progressed to end-stage renal disease, and 4 died during the follow-up period.
Conclusion:
MGN with crescents associated with ANCAs or anti-GBM antibodies is a rare dual glomerulopathy. Patients present with heavy proteinuria, microscopic hematuria, and acute kidney injury and should be treated for a rapidly progressive glomerulonephritis. Prognosis is variable, and 40% of patients progress to end-stage renal disease.
Insights
This study examines membranous glomerulonephritis (MGN) with necrotizing and crescentic glomerulonephritis (NCGN), a rare dual condition. Patients often experience severe kidney injury, with outcomes varying significantly, highlighting the need for prompt treatment.
Area of Science:
- Nephrology
- Immunopathology
- Glomerular Diseases
Background:
- Membranous glomerulonephritis (MGN) rarely co-occurs with necrotizing and crescentic glomerulonephritis (NCGN).
- This dual glomerulopathy is often associated with specific autoantibodies, including anti-neutrophil cytoplasm antibodies (ANCAs), anti-glomerular basement membrane (GBM) antibodies, and anti-phospholipase A2 receptor (PLA2R) antibodies.
Purpose of the Study:
- To report the clinical and pathological findings of patients with coexisting MGN and NCGN.
- To investigate the association of this rare condition with ANCA, anti-GBM, and anti-PLA2R antibodies.
- To analyze patient outcomes and treatment responses.
Main Methods:
- Retrospective analysis of 15 patients diagnosed with MGN and NCGN.
- Evaluation of clinical data, including autoantibody profiles (ANCA, anti-GBM, anti-PLA2R), urinalysis, and kidney biopsy findings.
- Assessment of treatment regimens (steroids, cyclophosphamide, rituximab) and patient follow-up data.
Main Results:
- The cohort (15 patients, median age 63) presented with heavy proteinuria and microscopic hematuria.
- MGN and NCGN were diagnosed concurrently in 12 patients; 7 had ANCA, 5 had anti-GBM, and 2 had anti-PLA2R antibodies.
- At median 72-month follow-up, 9 patients showed stabilized/improved renal function, 6 progressed to end-stage renal disease, and 4 died.
Conclusions:
- MGN with crescents associated with ANCAs or anti-GBM antibodies represents a rare dual glomerulopathy.
- Patients present with acute kidney injury, heavy proteinuria, and hematuria, requiring treatment for rapidly progressive glomerulonephritis.
- The prognosis is variable, with a significant proportion (40%) progressing to end-stage renal disease.
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