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Relapsing polychondritis: clinical presentations, disease activity and outcomes
Aman Sharma1, Arjun Dutt Law2, Pradeep Bambery3
1Department of Internal Medicine, Postgraduate Institute of medical education and research, Chandigarh, India. amansharma74@yahoo.com.
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.
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