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Clin J Am Soc Nephrol. 2009 Feb;4(2):299-308. doi: 10.2215/CJN.04060808. Epub 2009 Jan 21.

Membranous glomerulonephritis with ANCA-associated necrotizing and crescentic glomerulonephritis.

Author information

1
Department of Pathology, Columbia University, College of Physicians & Surgeons, 630 West 168th Street, VC14-224, New York, NY 10032, USA. sn386@columbia.edu

Abstract

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.

PMID:
19158367
PMCID:
PMC2637583
DOI:
10.2215/CJN.04060808
[Indexed for MEDLINE]
Free PMC Article

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