Case report of relapsing polychondritis in children

Background

Relapsing polychondritis (RP) is a rare, chronic, multisystem autoimmune disease. It is difficult to diagnose and characterized by recurrent episodes of cartilage inflamation in various tissue that potentially dangerous and life-threatening. This disease occurs in the 4^th or 5^th decade of life with average age is 51 years old, but only a very few cases of RP in children have been reported. Tissue containing cartilage that become inflammed include hyaline cartilage of nose and ears, joints, fibrocartilage in axial area, and tracheobronchial branches. Relapsing polychondritis can also cause inflammations in every structure that has rich proteoglicans, such as eyes, heart, and blood vessels which has biochemical structure resemble to that of cartilage. Pathogenesis of RP are suspected by the developing of autoantibody to the cartilage components, especially Collagen type II, that cause an inflammation process and cellular mechanism that involved lysosom release enzyme. The final result was cartilage destruction.        

Method

This is a case report of a 12-year-old boy with dyspnea as the chief complain, and a history of recurrent hoarseness and dyspnea since a month before admission. From the physical examination, there was a saddle nose deformity, increased ESR (40/125 mm/hour), positive ANA test with nuclear pattern, airway narrowing in larynx and pharynx that was showed in soft tissue neck photo, and laryngotracheomalacia in tracheoscopy.

Result

The patient was diagnosed as relapsing polychondritis (RP) and treated with prednison 2 mg/kg of bodyweight/day. The next bronchoscopy showed that the patient’s airway became narrowed, so we added immunosupressive agent (methotrexate), started from 10 mg/week. After the patient got methotrexate for 6 weeks, there was a clinical improvement and the tracheoscopy examination showed that the karina, the right and left prime bronchus was opened, and no evidence of granulation.

Conclusion

Steroids alone did not give good result as expected, but the combination with methotrexate in this patient was proved useful and gave better clinical improvement.

Key word: Collagen type II, laryngotracheomalacia, methotrexate, relapsing polychondritis