[Relapsing polychondritis with idiopathic hypoparathyroidism].

A 49-year-old man who had been treated with vitamin D since idiopathic hypoparathyroidism (IPH) was diagnosed at age 15 was referred to our hospital because of progressive wheezing. The same condition was also diagnosed in his sister at age 19. Chest radiography and computed tomography (CT) showed diffuse tracheal stenosis to a diameter of 6-8 mm. Laryngoscopy revealed no abnormality in the patient's epiglottis or vocal cords. No intact parathyroid hormone was detected in his sera but a brain CT scan showed calcification of the basal ganglia, which was compatible with IPH. Steroid therapy relieved his wheezing occasionally, but with little improvement of the narrowing of his trachea. He died of respiratory failure accompanied by lung abscess 15 weeks later. Autopsy revealed tracheal stenosis, thickening of the tracheal wall with marked fibrosis, and slight infiltration of inflammatory cells, which supported a diagnosis of relapsing polychondritis. This is the first report of relapsing polychondritis associated with IPH. Since both diseases are associated with autoimmune activity, this case may provide some insight into the pathogeneses of both diseases.