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

16 Citations (Scopus)

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

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Relapsing Polychondritis
India
Survival
Proteoglycans
Rare Diseases
Hearing Loss
Arthritis
Retrospective Studies
Observation
Inflammation
Skin

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

Relapsing polychondritis : clinical presentations, disease activity and outcomes. / Sharma, Aman; Law, Arjun D utt; Bambery, Pradeep; Sagar, Vinay; Wanchu, Ajay; Dhir, Varun; Vijayvergiya, Rajesh; Sharma, Kusum; Gupta, Ashok; Panda, Naresh K.; Singh, Surjit.

In: Orphanet Journal of Rare Diseases, Vol. 9, 2014, p. 198.

Research output: Contribution to journalArticle

Sharma, A, Law, ADU, Bambery, P, Sagar, V, Wanchu, A, Dhir, V, Vijayvergiya, R, Sharma, K, Gupta, A, Panda, NK & Singh, S 2014, 'Relapsing polychondritis: clinical presentations, disease activity and outcomes', Orphanet Journal of Rare Diseases, vol. 9, pp. 198. https://doi.org/10.1186/s13023-014-0198-1
Sharma, Aman ; Law, Arjun D utt ; Bambery, Pradeep ; Sagar, Vinay ; Wanchu, Ajay ; Dhir, Varun ; Vijayvergiya, Rajesh ; Sharma, Kusum ; Gupta, Ashok ; Panda, Naresh K. ; Singh, Surjit. / Relapsing polychondritis : clinical presentations, disease activity and outcomes. In: Orphanet Journal of Rare Diseases. 2014 ; Vol. 9. pp. 198.
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