Relapsing polychondritis
This is a rare autoinflammatory disorder which causes immune-mediated destruction of cartilage.
Clinical features include:
- Auricular cartilage is the most commonly affected, followed by nasal cartilage.
- Episcleritis and anterior scleritis commonly co-exist.
- Seronegative asymmetric polyarthritis affecting the small joints is a common presenting symptom.
- Laryngotracheal cartilage is affected in up to 50% and can present with cough, hoarseness or stridor. Other respiratory manifestations include recurrent lower respiratory tract infections.
- Cardiac involvement is the second most common cause of mortality after respiratory causes. Aortic regurgitation secondary to aortic root dilatation is the most common cardiac feature.
- Other systems, such as the kidneys, GI tract, and CNS can be involved, but this is rarer.
Treatment varies widely, from NSAIDs and colchicine to biologics such as anti-TNF agents, depending on severity.
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