Membranous glomerulonephritis with crescents
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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.
Keywords
IgG4 Membranous glomerulonephritis Necrotizing glomerulonephritis PLA2RNotes
Acknowledgments
We thank our Nephrology colleagues who provided detailed clinical and follow-up data: Doctors Pei-Li Wang, Leah Swetnam, Richard Kebler, Julie Raggio, Hem Deodhar, David Rozansky, Kendal Michaels, Jeanne Mowry, Kenneth Stringer, Dean Raniele, Clayton Smiley, and Shukri Osman. Part of this work was presented at the 2012 meeting of the United States and Canadian Academy of Pathology.
Conflict of interest
The authors declare that they have no conflict of interest.
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