Membranous glomerulonephritis with crescents

Caroline M.F. Barrett, Megan L. Troxell, Christopher P. Larse, Donald C. Houghton

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

Purpose: The coexistence of membranous glomerulonephritis (MGN) and necrotizing and crescentic glomerulonephritis (NCGN) is an unusual finding in a renal biopsy except in lupus nephritis. Little is known about whether these lesions are causally related in any clinical setting. Methods: We reviewed the pathology, presentation, and clinical course of 13 non-lupus patients with combined MGN and NCGN in native kidney biopsies (nine females, four males; median age 69 years), with particular attention to evidence of secondary MGN. Additional IgG subclass and phospholipase A2 receptor (PLA2R) immunofluorescence studies were conducted in seven cases. Results: Eight biopsies were pauci-immune other than the capillary wall deposits of MGN; one patient had a non-lupus immune complex disease, and four had mesangial deposits, including one with rare subendothelial deposits. None had anti-glomerular basement membrane disease. IgG4 was dominant or codominant in the capillary wall deposits in three cases and virtually absent in four; PLA2R was positive in two cases, and negative in five. Seven patients were judged to have secondary MGN, including five of eight ANCA+ patients. Twelve patients were treated with combinations of steroids, cyclophosphamide, rituximab, followed by durable response in seven and relentless progression to end stage renal disease in four. Conclusions: Secondary MGN occurs with higher frequency in ANCA-positive NCGN than in the general MGN population. A causal relationship between MGN and NCGN was not established in any patient, but circumstances suggest a common cause in several, including immune complex disease, drug reaction and paraneoplastic syndrome.

Original languageEnglish (US)
Pages (from-to)963-971
Number of pages9
JournalInternational Urology and Nephrology
Volume46
Issue number5
DOIs
StatePublished - Jan 1 2014

Keywords

  • IgG4
  • Membranous glomerulonephritis
  • Necrotizing glomerulonephritis
  • PLA2R

ASJC Scopus subject areas

  • Nephrology
  • Urology

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