Cargando…

Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders

Relapsing polychondritis (RPC) is an uncommon autoimmune systemic disease characterized by recurrent inflammation of the cartilage tissue. It can occur alone or in association with other autoimmune diseases, vasculitis, or hematologic disorders. However, the association of RPC with dermatomyositis i...

Descripción completa

Detalles Bibliográficos
Autores principales: Ríos-Rivera, Rafael A., Vilá, Luis M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10113061/
https://www.ncbi.nlm.nih.gov/pubmed/37082027
http://dx.doi.org/10.1155/2023/3719502
_version_ 1785027743719620608
author Ríos-Rivera, Rafael A.
Vilá, Luis M.
author_facet Ríos-Rivera, Rafael A.
Vilá, Luis M.
author_sort Ríos-Rivera, Rafael A.
collection PubMed
description Relapsing polychondritis (RPC) is an uncommon autoimmune systemic disease characterized by recurrent inflammation of the cartilage tissue. It can occur alone or in association with other autoimmune diseases, vasculitis, or hematologic disorders. However, the association of RPC with dermatomyositis is extremely rare. Herein, we present a case of a 38-year-old man who developed concurrent RPC and clinically amyopathic dermatomyositis (CADM) manifested by auricular chondritis, nasal chondritis, polyarthritis, gottron papules, fingertip papules, skin biopsy consistent with dermatomyositis, and positive antimelanoma differentiation-associated gene 5 (MDA5) antibodies. RPC features resolved with corticosteroids, but CADM manifestations were resistant to corticosteroids, cyclophosphamide, azathioprine, and hydroxychloroquine. Subsequent therapy with rituximab was effective to control CADM manifestations. This case highlights the importance of recognizing CADM as part of the autoimmune diseases linked with RPC and maintaining a high level of awareness to initiate effective therapy to avoid the long-term complications associated with these conditions.
format Online
Article
Text
id pubmed-10113061
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Hindawi
record_format MEDLINE/PubMed
spelling pubmed-101130612023-04-19 Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders Ríos-Rivera, Rafael A. Vilá, Luis M. Case Rep Rheumatol Case Report Relapsing polychondritis (RPC) is an uncommon autoimmune systemic disease characterized by recurrent inflammation of the cartilage tissue. It can occur alone or in association with other autoimmune diseases, vasculitis, or hematologic disorders. However, the association of RPC with dermatomyositis is extremely rare. Herein, we present a case of a 38-year-old man who developed concurrent RPC and clinically amyopathic dermatomyositis (CADM) manifested by auricular chondritis, nasal chondritis, polyarthritis, gottron papules, fingertip papules, skin biopsy consistent with dermatomyositis, and positive antimelanoma differentiation-associated gene 5 (MDA5) antibodies. RPC features resolved with corticosteroids, but CADM manifestations were resistant to corticosteroids, cyclophosphamide, azathioprine, and hydroxychloroquine. Subsequent therapy with rituximab was effective to control CADM manifestations. This case highlights the importance of recognizing CADM as part of the autoimmune diseases linked with RPC and maintaining a high level of awareness to initiate effective therapy to avoid the long-term complications associated with these conditions. Hindawi 2023-04-11 /pmc/articles/PMC10113061/ /pubmed/37082027 http://dx.doi.org/10.1155/2023/3719502 Text en Copyright © 2023 Rafael A. Ríos-Rivera and Luis M. Vilá. 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
Ríos-Rivera, Rafael A.
Vilá, Luis M.
Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title_full Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title_fullStr Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title_full_unstemmed Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title_short Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders
title_sort coexistent relapsing polychondritis and clinically amyopathic dermatomyositis: a rare association of autoimmune disorders
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10113061/
https://www.ncbi.nlm.nih.gov/pubmed/37082027
http://dx.doi.org/10.1155/2023/3719502
work_keys_str_mv AT riosriverarafaela coexistentrelapsingpolychondritisandclinicallyamyopathicdermatomyositisarareassociationofautoimmunedisorders
AT vilaluism coexistentrelapsingpolychondritisandclinicallyamyopathicdermatomyositisarareassociationofautoimmunedisorders