Relapsing polychondritis in North India: a report of 10 patients

Abstract

Objective: Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We describe the outcome of 10 patients followed up at the Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, over the past 10 years.

Methods: All the patients fulfilling the diagnostic criteria suggested by McAdam et al (Medicine (Baltimore) 1976;55:193-215) and modified by Damiani and Levine (Laryngoscope 1979;89;929-46) were included in the study. Detailed clinical features, investigations, treatment given, and outcome were recorded on file.

Results: Six women and four men, mean age 48.1 years (range 26-65 years), met the criteria for diagnosis. The mean duration of symptoms, before diagnosis, was 27 months (range 1-72 months). Clinical features included auricular chondritis (100%), arthritis (80%), fever (50%), constitutional symptoms (50%), eye involvement (50%), hearing loss (40%), collapsed bridge of nose (30%), laryngotracheal involvement (20%), aortic dilatation (10%), and nephrotic syndrome (10%). Two patients had the MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome. The number of episodes of cartilaginous inflammation varied from one to eight. Treatment included oral prednisolone (n = 9), intermittent 'pulse' cyclophosphamide (n = 2), and azathioprine (n = 2). One patient required tracheostomy and died later. The others are doing well. Mean duration of follow-up was 35.5 months (range 1-79 months).

Conclusions: The diagnosis of this potentially lethal condition is frequently delayed. Our series suggests that clinical manifestations of RP are similar in Caucasian, Oriental, and Asian populations. Laryngotracheal involvement was seen less frequently in our patients.