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 journalArticlepeer-review

23 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
StatePublished - 2014

ASJC Scopus subject areas

  • Genetics(clinical)
  • Pharmacology (medical)

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