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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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Autores principales: Mereniuk, Eric, Sabbah, Laura, Makhzoum, Jean-Paul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8133855/
https://www.ncbi.nlm.nih.gov/pubmed/34055432
http://dx.doi.org/10.1155/2021/9940293
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author Mereniuk, Eric
Sabbah, Laura
Makhzoum, Jean-Paul
author_facet Mereniuk, Eric
Sabbah, Laura
Makhzoum, Jean-Paul
author_sort Mereniuk, Eric
collection PubMed
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 pubmed-81338552021-05-27 A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid Mereniuk, Eric Sabbah, Laura Makhzoum, Jean-Paul Case Rep Nephrol Case Report 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. Hindawi 2021-05-11 /pmc/articles/PMC8133855/ /pubmed/34055432 http://dx.doi.org/10.1155/2021/9940293 Text en Copyright © 2021 Eric Mereniuk et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Mereniuk, Eric
Sabbah, Laura
Makhzoum, Jean-Paul
A Case of Severe and Refractory Membranous Nephropathy Associated to Mucous Membrane Pemphigoid
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
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8133855/
https://www.ncbi.nlm.nih.gov/pubmed/34055432
http://dx.doi.org/10.1155/2021/9940293
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