Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders.
Rafael A Ríos-RiveraLuis M ViláPublished in: Case reports in rheumatology (2023)
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.
Keyphrases
- interstitial lung disease
- disease activity
- systemic sclerosis
- multiple sclerosis
- rheumatoid arthritis
- idiopathic pulmonary fibrosis
- systemic lupus erythematosus
- oxidative stress
- low dose
- squamous cell carcinoma
- high dose
- cell proliferation
- risk factors
- gene expression
- bone marrow
- radiation therapy
- copy number
- diffuse large b cell lymphoma
- ultrasound guided
- cell therapy
- signaling pathway
- extracellular matrix