A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid

Primary membranous nephropathy (MN) and mucous membrane pemphigoid (MMP) are two autoimmune conditions with well-defined diagnostic and treatment guidelines. MN has been linked to bullous pemphigoid (BP) in certain case reports, though little is known regarding the association of MN and other bullou...

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Main Authors: Eric Mereniuk, Laura Sabbah, Jean-Paul Makhzoum
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Nephrology
Online Access:http://dx.doi.org/10.1155/2021/9940293
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author Eric Mereniuk
Laura Sabbah
Jean-Paul Makhzoum
author_facet Eric Mereniuk
Laura Sabbah
Jean-Paul Makhzoum
author_sort Eric Mereniuk
collection DOAJ
description Primary membranous nephropathy (MN) and mucous membrane pemphigoid (MMP) are two autoimmune conditions with well-defined diagnostic and treatment guidelines. MN has been linked to bullous pemphigoid (BP) in certain case reports, though little is known regarding the association of MN and other bullous diseases. The association of MN and MMP has rarely been described, and very little data exist regarding the treatment of this association. We report a case of severe refractory membranous nephropathy secondary to mucous membrane pemphigoid successfully treated with rituximab. A 35-year-old woman with known MMP was referred to our clinic for new-onset generalized edema and proteinuria. MN was confirmed on renal biopsy. Despite therapy with high-dose systemic glucocorticoids, combined with mycophenolate mofetil, and later azathioprine, nephrotic-range proteinuria persisted even at a daily dose of prednisone of 40 mg. The patient was then started on rituximab infusions, which induced remission of both mucous membrane pemphigoid and membranous glomerulonephritis. This suggests that MN can be secondary to MMP, and rituximab may be useful induce remission in cases that are refractory to standard therapy.
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spelling doaj-art-5150f9addc63445abe67cb4c8ff23bb92025-02-03T06:07:40ZengWileyCase Reports in Nephrology2090-66412090-665X2021-01-01202110.1155/2021/99402939940293A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane PemphigoidEric Mereniuk0Laura Sabbah1Jean-Paul Makhzoum2Department of Medicine, Université de Montreal, Montreal, CanadaDivision of Dermatology, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Université de Montreal, Montreal, CanadaVasculitis Clinic, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Université de Montreal, Montreal, CanadaPrimary membranous nephropathy (MN) and mucous membrane pemphigoid (MMP) are two autoimmune conditions with well-defined diagnostic and treatment guidelines. MN has been linked to bullous pemphigoid (BP) in certain case reports, though little is known regarding the association of MN and other bullous diseases. The association of MN and MMP has rarely been described, and very little data exist regarding the treatment of this association. We report a case of severe refractory membranous nephropathy secondary to mucous membrane pemphigoid successfully treated with rituximab. A 35-year-old woman with known MMP was referred to our clinic for new-onset generalized edema and proteinuria. MN was confirmed on renal biopsy. Despite therapy with high-dose systemic glucocorticoids, combined with mycophenolate mofetil, and later azathioprine, nephrotic-range proteinuria persisted even at a daily dose of prednisone of 40 mg. The patient was then started on rituximab infusions, which induced remission of both mucous membrane pemphigoid and membranous glomerulonephritis. This suggests that MN can be secondary to MMP, and rituximab may be useful induce remission in cases that are refractory to standard therapy.http://dx.doi.org/10.1155/2021/9940293
spellingShingle Eric Mereniuk
Laura Sabbah
Jean-Paul Makhzoum
A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
Case Reports in Nephrology
title A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
title_full A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
title_fullStr A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
title_full_unstemmed A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
title_short A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
title_sort case of severe and refractory membranous nephropathy associated to mucous membrane pemphigoid
url http://dx.doi.org/10.1155/2021/9940293
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