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Inflammatory destructive lesions of cartilaginous structures, principally the ears, nose, trachea, larynx, and chest wall cartilage
Associated conditions include
ANCA vasculitis
Systemic lupus erythematosus
Rheumatoid arthritis
Autoimmune (Hashimoto) thyroiditis
Cancer (especially plasma cell myeloma)
Hematologic disorders (myelodysplastic syndrome)
Episodic and affects males and females equally
There are no serologic markers for relapsing polychondritis
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The cartilage is painful, swollen, and tender during an attack
Subsequently becomes atrophic, resulting in permanent deformity
Noncartilaginous manifestations
In 85% of patients, a migratory, asymmetric, and seronegative arthropathy occurs, affecting both large and small joints and the costochondral junctions
Laryngotracheal and bronchial chondritis can lead to life-threatening airway narrowing and collapse
Large vessel vasculitis is a frequently overlooked but potentially catastrophic complication
Differential diagnosis
Other causes of ear deformity (skin cancer, cauliflower ear [cartilage dissolution posttrauma], chondritis or perichondritis, chondrodermatitis nodularis helicis)
Other causes of nose deformity (syphilis, rhinophyma [rosacea], Hansen disease [leprosy], granulomatosis with polyangiitis)
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), a newly described X-linked genetic syndrome caused by somatic mutations in UBA1 in hematopoietic progenitor cells, especially in the presence of unexplained macrocytosis and evidence of systemic inflammation (high ESR/CRP)
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Prednisone, 0.5–1.0 mg/kg/day orally, is often effective
Dapsone, 100–200 mg/day orally, or methotrexate (7.5–20 mg/week orally) may have efficacy, sparing the need for long-term high-dose corticosteroid treatment
Involvement of the tracheobronchial tree may respond to TNF inhibitors