Relapsing polychondritis: Clinical presentations, disease activity and outcomes

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).

Patient Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis

“…RP is defined by inflammation of cartilaginous structures, and the disease course and clinical manifestations are highly variable. End-organ complications due to inflammation of cartilage and proteoglycanrich structures are diverse and include deformity of the ears (2), nose (3) and chest (4), subglottic stenosis (5), obstructive lung disease, tracheomalacia (6), bronchomalacia (7), recurrent pneumonia (8), mitral and aortic regurgitation (9,10), large and medium vessel aneurysms (11), blindness (12), deafness (13), renal insufficiency (14), chronic pain, and sudden death (15)(16)(17). The protean aspects of RP pose diagnostic challenges.…”

Relapsing Polychondritis: An Otolaryngologist's Perspective

“…14 The disease usually has a fluctuating, but progressive course, and the mainstay of treatment is suppression of autoimmunity and inflammation. 5 However, the outcome may be difficult to predict. The severity and chronicity of the disease, as well as the extent of multisystemic involvement, determine the choice of pharmacological agent used, as well as the variable outcomes that are reported in literature.…”

“…The severity and chronicity of the disease, as well as the extent of multisystemic involvement, determine the choice of pharmacological agent used, as well as the variable outcomes that are reported in literature. 1,2,4,5 Often, the choice of treatment is individualized. Nonsteroidal anti-inflammatory drugs form the first line of management in uncomplicated cases, with only nasal or auricular cartilage involvement.…”

“…Airway involvement is seen in nearly half the cases of RP in Caucasians, 3,4 whereas studies from India have reported prevalence ranging from 12 to 43%. 5,6 10.5005/jp-journals-10003-1254…”

Image Gallery: Relapsing polychondritis associated with monoclonal gammopathy

“…The histological findings were compatible with relapsing polychondritis (RP). RP is a recurring, progressive, autoimmune, inflammatory disorder that affects proteoglycan‐rich tissues . It has been described as a paraneoplastic phenomenon .…”