Abstract
Relapsing polychondritis is a rare cartilaginous inflammatory disease affecting the external ear, nose, peripheral joints and tracheobronchial tree. It is characterized by recurrent inflammation and degeneration of cartilage and connective tissue. A 72-year-old man complained of dyspnea, cough and wheezing for 2 months. Diffuse wall thickening and narrowing from the trachea to segmental bronchus were seen on chest CT. Tracheostomy was performed in order to avoid as-phyxia, and he was diagnosed as relapsing polychondritis on the basis of pathology evaluation of a tracheal biopsy specimen. He was treated with high doses of a glucocorticoid, with which his symptoms improved. However, the cough and wheezing recurred after tapering of the glucocorticoid. His symptoms thereafter were improved by combination of the glucocorticoid with cyclosporine. The immunosuppressive agent provided effective treatment for glucocorticoid-resistant relapsing polychondritis.
Highlights
Relapsing polychondritis (RP) is a rare disorder with an estimated incidence of 3 per million populations [1] [2]
A computed tomography (CT) scan repeated at 6 months after initiating treatment showed a further decrease in stenosis of the trachea and bronchi (Figure 1(e), Figure 1(f))
RP is characterized by inflammation of cartilage, serum C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR) are increased in correlation with disease activity [6]
Summary
Relapsing polychondritis (RP) is a rare disorder with an estimated incidence of 3 per million populations [1] [2]. RP is reportedly related to autoimmunity, and involves the external ear, nose, peripheral joints and tracheobron-. (2015) A Case of Relapsing Polychondritis Successfully Treated with Combination of a Glucocorticoid and Cyclosporine. In cases with involvement of the tracheobronchial cartilage, symptoms such as non-productive cough, wheezing and dyspnea occur, fatal asphyxia has been reported [4]. Immunosuppressive agents are used in combination with glucocorticoids in patients with a poor response to the glucocorticoid [6]. Standard regimens of immunosuppressive agents have not been established. We report a case of airway-limiting RP that was successfully treated by combination of a glucocorticoid and cyclosporine
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