Relapsing polychondritisICD-11 Subcategory

Relapsing polychondritis is a multisystem inflammatory disease of unknown etiology affecting the cartilage. The disease is characterized by intermittent or fluctuant inflammatory manifestations due to inflammation of the cartilaginous structures, resulting in tissue damage and tissue destruction. Chondritis of auricular, nasal, tracheal cartilage predominates in this disease, suggesting response to tissue-specific antigens such as collagen II and cartilage matrix protein (matrillin-1). In about one third of patients, RP is associated with vasculitis (from isolated cutaneous leucocytoclastic vasculitis to systemic polyangiitis) and autoimmune rheumatic diseases (autoimmune rheumatic diseases mainly rheumatoid arthritis and systemic lupus erythematosus). Hematological malignant diseases, gastrointestinal disorders and endocrine diseases may also occur. Functional and anatomical evaluation for upper and lower airway disease is essential in evaluation and management of the disease.