Relapsing polychondritis (RP) is a rare multisystemic disease widely accepted as a complex autoimmune disorder affecting proteoglycan-rich structures and cartilaginous tissues, including the ear, nose, larynx, trachea, bronchi, peripheral joints, eye, heart, and skin, with high risk of misdiagnosis. The clinical spectrum may vary from intermittent inflammatory episodes leading to unesthetic structural deformities to life-threatening cardiopulmonary manifestations, such as airway collapse and valvular regurgitation. The diagnosis of RP is largely based on the clinical features and the role of laboratory investigations is purely supportive and to rule out other related or associated systemic diseases. Treatment in RP is largely symptomatic and a standard management protocol is yet to be established due to its rarity.
Category
Musculoskeletal disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
15 Diseases of the musculoskeletal system or connective tissue
Osteopathies or chondropathies
FB82 Chondropathies
H01758 Relapsing polychondritis
