TY - JOUR
T1 - Relapsing polychondritis
T2 - clinical presentations, disease activity and outcomes
AU - Sharma, Aman
AU - Law, Arjun D.utt
AU - Bambery, Pradeep
AU - Sagar, Vinay
AU - Wanchu, Ajay
AU - Dhir, Varun
AU - Vijayvergiya, Rajesh
AU - Sharma, Kusum
AU - Gupta, Ashok
AU - Panda, Naresh K.
AU - Singh, Surjit
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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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U2 - 10.1186/s13023-014-0198-1
DO - 10.1186/s13023-014-0198-1
M3 - Article
C2 - 25527201
AN - SCOPUS:84964696360
SN - 1750-1172
VL - 9
SP - 198
JO - Orphanet Journal of Rare Diseases
JF - Orphanet Journal of Rare Diseases
ER -