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Published ahead of print on January 21, 2009
Clin J Am Soc Nephrol 4: 299-308, 2009
© 2009 American Society of Nephrology
doi: 10.2215/CJN.04060808

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Clinical Immunology and Pathology

Membranous Glomerulonephritis with ANCA-Associated Necrotizing and Crescentic Glomerulonephritis

Samih H. Nasr*, Samar M. Said*, Anthony M. Valeri{dagger}, Michael B. Stokes*, Naveed N. Masani{ddagger}, Vivette D. D'Agati*, and Glen S. Markowitz*

* Department of Pathology, Columbia University, College of Physicians & Surgeons, New York, New York; {dagger} Department of Medicine, Division of Nephrology, Columbia University, College of Physicians & Surgeons, New York, New York; and {ddagger} Department of Medicine, Winthrop University Hospital, Mineola, New York

Correspondence: Dr. Samih H. Nasr, Department of Pathology, Columbia University, College of Physicians & Surgeons, 630 West 168th Street, VC14–224, New York, NY 10032. Phone: 212-305-7460; Fax: 212-342-5380; E-mail: sn386{at}columbia.edu

Background and objectives: Only rare cases of concurrent membranous glomerulonephritis (MGN) and antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis (NCGN) have been reported.

Design, setting, participants, & measurements: The authors report the clinical and pathologic findings in 14 patients with MGN and ANCA-associated NCGN.

Results: The cohort consisted of eight men and six women with a mean age of 58.7 yr. ANCA positivity was documented by indirect immunofluorescence or ELISA in all patients. Indirect immunofluorescence was positive in 13 patients (seven P-ANCA, five C-ANCA, one atypical ANCA). ELISA was positive in nine of 10 patients (five MPO-ANCA, three PR3-ANCA, one MPO- and PR3-ANCA). Clinical presentation included heavy proteinuria (mean 24-hr urine protein 6.5 g/d), hematuria, and acute renal failure (mean creatinine 4.4 mg/dl). Pathologic evaluation revealed MGN and NCGN, with crescents involving a mean of 32% of glomeruli. On ultrastructural evaluation, the majority of cases showed stage I or II membranous changes. Follow-up was available for 13 patients, 12 of whom were treated with steroids and cyclophosphamide. At a mean follow-up of 24.3 mo, five patients progressed to ESRD, seven had stabilization or improvement in renal function, and one had worsening renal function. Five patients, including three with ESRD, died during the follow-up period. The only independent predictor of progression to ESRD was serum creatinine at biopsy.

Conclusions: MGN with ANCA-associated NCGN is a rare dual glomerulopathy seen in patients with heavy proteinuria, acute renal failure, and active urine sediment. Prognosis is variable, with 50% of patients reaching endpoints of ESRD or death.




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K. Matsumoto, H. Honda, T. Shibata, D. Sanada, Y. Wada, E. Ashikaga, A. Kuroki, K. Kitazawa, and T. Akizawa
MPO-ANCA crescentic glomerulonephritis complicated by membranous nephropathy: MPO demonstrated in epimembranous deposits
NDT Plus, December 1, 2009; 2(6): 461 - 465.
[Abstract] [Full Text] [PDF]




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