Title |
Relapsing polychondritis: clinical presentations, disease activity and outcomes
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Published in |
Orphanet Journal of Rare Diseases, December 2014
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DOI | 10.1186/s13023-014-0198-1 |
Pubmed ID | |
Authors |
Aman Sharma, Arjun Dutt Law, Pradeep Bambery, Vinay Sagar, Ajay Wanchu, Varun Dhir, Rajesh Vijayvergiya, Kusum Sharma, Ashok Gupta, Naresh K Panda, Surjit Singh |
Abstract |
BackgroundRelapsing 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.MethodsA 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.ResultsTen 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).ConclusionsApart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Canada | 1 | 17% |
United States | 1 | 17% |
United Kingdom | 1 | 17% |
Saudi Arabia | 1 | 17% |
Unknown | 2 | 33% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 5 | 83% |
Scientists | 1 | 17% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Canada | 1 | 3% |
Unknown | 30 | 97% |
Demographic breakdown
Readers by professional status | Count | As % |
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Other | 7 | 23% |
Student > Doctoral Student | 4 | 13% |
Researcher | 4 | 13% |
Professor | 3 | 10% |
Professor > Associate Professor | 3 | 10% |
Other | 6 | 19% |
Unknown | 4 | 13% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 18 | 58% |
Nursing and Health Professions | 3 | 10% |
Pharmacology, Toxicology and Pharmaceutical Science | 1 | 3% |
Immunology and Microbiology | 1 | 3% |
Chemistry | 1 | 3% |
Other | 0 | 0% |
Unknown | 7 | 23% |