Polychondritis

Polychondritis, most commonly referred to as relapsing polychondritis, is a rare autoimmune disorder characterized by recurrent inflammation of cartilaginous structures and other tissues. The exact cause is unknown, but an immune-mediated process directed against cartilage components, such as type II collagen, is suspected. The condition can affect individuals at any age but is most often diagnosed in adults.

Clinical features frequently involve the ears, nose, airways, joints, eyes, and skin. Auricular chondritis presents with painful, red, swollen ears while sparing the earlobe. Nasal chondritis can lead to a saddle-nose deformity. Respiratory tract involvement affects laryngeal and tracheal cartilage, potentially causing cough, hoarseness, wheezing, or airway obstruction. Inflammatory arthritis is common and may be nonerosive. Ocular inflammation, such as episcleritis or uveitis, occurs in a significant minority. Other manifestations can include skin lesions, hearing loss, or cardiovascular and neurological complications.

Diagnosis relies on a combination of clinical features, supported by biopsy of affected cartilage showing inflammatory infiltrates and loss of cartilage structure. Laboratory tests are nonspecific, often showing elevated inflammatory markers (ESR, CRP). There is no single diagnostic test; diagnostic criteria (such as those proposed by McAdam) are used to guide assessment, alongside imaging and exclusion of mimicking conditions.

Treatment aims to control inflammation and prevent damage. Systemic corticosteroids are the cornerstone of therapy, with steroid-sparing agents such as methotrexate, azathioprine, or mycophenolate used for sustained disease or relapses. More advanced or refractory cases may require biologic therapies (e.g., TNF inhibitors, rituximab, or IL-6 inhibitors) and airway management for laryngotracheal involvement. The course is variable, with relapses common, and prognosis depends on the extent of organ involvement and response to therapy.