glycogen and Polychondritis--Relapsing

A 57-year-old man and a 70-year-old woman with relapsing polychondritis are reported. The man, suffering from arthralgias, respiratory obstruction, external ear and sanddle-nose deformities, conjunctivitis and irido-cyclitis, died after 4 years from airway obstruction because of tracheal and bronchial collapse. The woman is alive 8 months after the development of respiratory obstruction, probably caused by radiographically demonstrated tracheal obstruction, a saddle-nose deformity and hearing impairment. Microscopically, the involved cartilages showed degenerative and slight inflammatory changes and were eventually replaced by fibrous tissue. Histochemical studies, utilizing staining with Alcian blue at controlled electrolyte concentrations (Scott technique) and at controlled pH:s, with or without digestion with bacterial chondroitinase ABC; and staining with the PAS-method, with or without diastase digestion, revealed a complete or relative loss of glucosaminoglycans and glycogen. A biosynthetic defect is considered unlikely to be the primary pathogenetic mechanism of relapsing polychondritis. Histological and histochemical examination of biopsies from involved cartilages contribute to a definite diagnosis.

Topics: Aged; Alcian Blue; Aminosalicylic Acid; Amylases; Cartilage; Chondroitinases and Chondroitin Lyases; Electrolytes; Female; Glycogen; Glycosaminoglycans; Histocytochemistry; Humans; Hydrogen-Ion Concentration; Male; Middle Aged; Polychondritis, Relapsing