Relapsing Polychondritis Presenting 2 Years after Systemic Sclerosis with Pulmonary Arterial Hypertension

Relapsing polychondritis (RPC) is a systemic immune-mediated disease characterized by recurrent and progressive inflammation of cartilaginous tissues. 64% of RPC patients concurrent with other autoimmune disorders, there are very few reports about the concomitant RPC patients with systemic sclerosis...

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Detalles Bibliográficos
Autores principales: Su, Li, Zhang, Na, Wang, Hui, Yang, Zhenwen, Wei, Wei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sciendo 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9524778/
https://www.ncbi.nlm.nih.gov/pubmed/36465970
http://dx.doi.org/10.2478/rir-2021-0016
Descripción
Sumario:Relapsing polychondritis (RPC) is a systemic immune-mediated disease characterized by recurrent and progressive inflammation of cartilaginous tissues. 64% of RPC patients concurrent with other autoimmune disorders, there are very few reports about the concomitant RPC patients with systemic sclerosis (SSc). Herein we report a case of RPC in a 50-year-old female following SSc with pulmonary arterial hypertension (PAH) 2 years ago. She was treated with corticosteroids, immunosuppressive drugs, oral endothelin-A receptor antagonist and phosphodiesterase type 5 inhibitors. Her ocular and auricular symptoms disappeared quickly. The hemodynamic parameters were also significantly improved after treatment. To our knowledge, this is the first RPC complicated with SSc-PAH reported.

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