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Relapsing polychondritis: clinical presentations, disease activity and outcomes
Aman Sharma1, Arjun Dutt Law2, Pradeep Bambery3
1Department of Internal Medicine, Postgraduate Institute of medical education and research, Chandigarh, India. amansharma74@yahoo.com.
Relapsing polychondritis (RP) in India presents similarly to global patterns, with auricular chondritis and arthritis being common. This study offers insights into RP patient experiences in a North Indian tertiary center.
Area of Science:
- Rheumatology
- Immunology
- Rare Diseases
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by progressive inflammation of cartilaginous tissues.
- Limited single-center case series exist globally, necessitating further research into regional disease characteristics.
Observation:
- This study retrospectively analyzed 26 patients diagnosed with relapsing polychondritis (RP) at a tertiary care center in North India.
- Patients met Damiani and Levine's modification of McAdam's diagnostic criteria.
- Data collected included clinical manifestations, disease activity (RPDAI), treatments, and outcomes.
Findings:
- The cohort comprised 10 males and 16 females with a median age of 45 years.
- Common manifestations included auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), and dermal involvement (26%).
- Cardiovascular and laryngotracheal involvement were observed in 11% of patients. Median RPDAI was 31, with an overall survival rate of 92.3% over a median of 13.5 years.
Implications:
- Relapsing polychondritis (RP) in India shares clinical similarities with international cohorts, except for a potentially lower incidence of laryngotracheal involvement.
- Findings contribute to understanding the epidemiology and clinical spectrum of RP in the Indian subcontinent.
- This data can inform clinical management and future research directions for relapsing polychondritis (RP).
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