Published in
BioMed Central, Orphanet Journal of Rare Diseases, 1(9), 2014
DOI: 10.1186/s13023-014-0198-1
Elsevier, Indian Journal of Rheumatology, (9), p. S9-S10
DOI: 10.1016/j.injr.2014.10.012
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- ORCID
- BioMed Central | PDF
- Elsevier
- [www.ncbi.nlm.nih.gov] | PDF
- [espace.library.uq.edu.au]
- [www.ojrd.com] | PDF
- [europepmc.org] | PDF
- [www.researchgate.net] | PDF
- [www.researchgate.net] | PDF
- [ojrd.biomedcentral.com] | PDF
- [www.ncbi.nlm.nih.gov] | PDF
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Relapsing polychondritis: clinical presentations, disease activity and outcomes
,
Vinay Sagar,
Pradeep Bambery,
Ajay Wanchu,
Varun Dhir,
Rajesh Vijayvergiya,
Kusum Sharma,
Ashok Gupta,
Naresh Kumar Panda,
Surjit Singh
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Abstract
Abstract 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.