Recurrent refractory polychondritis: A case report

Abstract

Relapsing polychondritis (RP) is an immune-mediated systemic disease characterized by recurrent attacks of inflammation of cartilage and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment in the affected areas. The most common clinical features include ear and nasal chondritis, as well as polyarthritis, though potentially all cartilage areas can be affected. Delay in diagnosis is frequently encountered due to the progression of the disease and non-specific symptoms at the beginning. 30% of RP patients may have concomitant autoimmune disorders. In our case, a 42-year-old male patient diagnosed with RP was treated with methotrexate, leflunomide, tumor necrosis factor inhibitors and tociluzumab, but showed no improvement. Consequently, the patient was administered cyclophosphamide. Unfortunately, pneumomediastinum developed on the sixth day of treatment. The condition resolved without the need for surgical intervention. However, although the case received many treatments mentioned in the literature, he still did not go into complete remission.

Keywords

• relapsing polychondritis
• pneumomediastinum
• cyclophosphamide