Relapsing polychondritis: clinical presentations, disease activity and outcomes (Orphanet Journal of Rare Diseases)

Background: Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India. Methods: A retrospective study with all patients meeting Damiani and Levine?s modification of McAdam?s diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index(RPDAI)], treatment and outcomes were recorded. Results: Ten men and sixteen women (median age 45?years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular(42%), dermal (26%), cardiovascular(11%) and laryngotracheal involvement(11%)characterized the clinical presentations. The median RPDAI was 31(range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5?years). Conclusions: Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.