Relapsing polychondritis: clinical presentations, disease activity and outcomes

Orphanet J Rare Dis. 2014 Dec 20:9:198. doi: 10.1186/s13023-014-0198-1.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Polychondritis, Relapsing / diagnosis*
  • Polychondritis, Relapsing / therapy*
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult

Substances

  • Immunosuppressive Agents