Relapsing polychondritis: clinical presentations, disease activity and outcomes

Aman Sharma, Arjun D utt Law, Pradeep Bambery, Vinay Sagar, Ajay Wanchu, Varun Dhir, Rajesh Vijayvergiya, Kusum Sharma, Ashok Gupta, Naresh K. Panda, Surjit Singh

Research output: Contribution to journalArticle

20 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)198
Number of pages1
JournalOrphanet Journal of Rare Diseases
Volume9
DOIs
Publication statusPublished - 2014

    Fingerprint

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Sharma, A., Law, A. D. U., Bambery, P., Sagar, V., Wanchu, A., Dhir, V., ... Singh, S. (2014). Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet Journal of Rare Diseases, 9, 198. https://doi.org/10.1186/s13023-014-0198-1