Relapsing polychondritis: clinical presentations, disease activity and outcomes.

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

doi:10.1186/s13023-014-0198-1

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.

Highlights

Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures
The data of all patients diagnosed with relapsing polychondritis registered with the Rheumatology department from 1997 – 2014 was collected and analysed
Twenty six patients who met the diagnostic criteria defined by McAdam and modified by Damiani and Levine were included in this analysis [1,8]

Summary

Introduction

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. Relapsing Polychondritis (RP,Orpha 728) is usually a recurring, autoimmune, inflammatory disorder predominantly affecting cartilage and other proteoglycan rich structures, with clinically significant ear, nose, articular and tracheobronchial tree involvement. The eyes, heart and blood vessels may be involved to a variable degree. Rare, it may be associated with significant morbidity and mortality especially if associated with respiratory tract or cardiovascular involvement. Smaller series from south-east Asia and India have, depicted clinical presentations at variance from those in Caucasian

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