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


METHODS
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.
Nasr
Samih H
SH
Department of Pathology, Columbia University, College of Physicians & Surgeons, 630 West 168th Street, VC14-224, New York, NY 10032, USA. sn386@columbia.edu
Said
Samar M
SM
Valeri
Anthony M
AM
Stokes
Michael B
MB
Masani
Naveed N
NN
D'Agati
Vivette D
VD
Markowitz
Glen S
GS
D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology / Q000473:pathology; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001706:Biopsy; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006417:Hematuria / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000276:immunology / Q000473:pathology; D007677:Kidney Function Tests; D007678:Kidney Glomerulus / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D011507:Proteinuria / Q000209:etiology / Q000276:immunology / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide; D003404:Creatinine