Relapsing polychondritis: report of an unusual case and a review of the literature

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Abstract
Relapsing polychondritis is an uncommon multisystem disorder, characterized by recurrent inflammation of cartilage at multiple sites, which can go unrecognized until classical signs of cartilage destruction occur. Destruction of the cartilage of the external ears and nose is easy to recognize. Central airway involvement is more difficult to recognize, but more important in terms of prognosis. Neurological symptoms and signs are uncommon, have been infrequently reported and may lead to incorrect diagnosis and delay in treatment. A case of relapsing polychondritis presenting as an acute encephalitis is described. The use of computed tomography (CT) in determining the presence and severity of central airway involvement is discussed. A 58-year-old man was admitted in June 1988 with a 6-week history of ear infection not responding to three courses of antibiotics. During this time he had been unwell with earache, headache, fatigue and lethargy and was sleeping for long periods. He also had fleeting arthralgia and intermittent fever with rigors. Two days before admission he deteriorated and became confused, uncooperative and drowsy. He was known to have been under the care of an ophthalmologist with left episcleritis, for the previous 8 months. In 1972, mild asthma was treated with inhaled bronchodilators and in 1983 he experienced costochondral chest pain. On admission he was found to be drowsy, confused and agitated. Bilateral conjunctivitis, swollen eyelids and tender pinnae were noted but the tympanic membranes were normal.