Relapsing Polychondritis
Top Contributors - Pacifique Dusabeyezu, Kim Jackson and Carina Therese Magtibay
Introduction[edit | edit source]
Relapsing polychondritis (RP) is a rare multisystem autoimmune condition with an unknown cause. It is characterized by recurring episodes of inflammation that lead to the gradual destruction of various cartilaginous tissues in the body. This includes elastic cartilage found in the ears and nose, hyaline cartilage in peripheral joints, fibrocartilage in the spine, and cartilage in the tracheobronchial system. RP can affect structures rich in proteoglycans, such as those in the eye, heart, blood vessels, and inner ear.[1]
Etiology[edit | edit source]
The etiology of Relapsing polychondritis remains unknown so far, and the pathogenesis appears to be mediated by an autoimmune reaction to type II collagen, which is abundant in cartilage and the sclera.[2]
Clinical Presentation[edit | edit source]
Epidemiology[edit | edit source]
RPC affects 1 in 1.4 million people per year in the UK, with a standardized mortality ratio of 2.16. Onset may be sudden, although in mild cases it can be insidious. Peak incidence is in the fifth decade of life (40–55 years old), but the disease has been described in young children and the very elderly.[2] Relapsing polychondritis (RP) primarily affects middle-aged adults, with a slight tendency towards affecting more women than men. In the United States, RP has an estimated annual incidence of 3.5 cases per one million people in the population, and among Department of Defense beneficiaries, the prevalence is approximately 4.5 cases per ten million individuals.[3]
Diagnostic Procedures[edit | edit source]
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Outcome Measures[edit | edit source]
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Management / Interventions
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Differential Diagnosis
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Prognosis[edit | edit source]
Most individuals with RP typically experience a variable disease course marked by intermittent episodes of inflammation. During each flare-up, the specific sites affected and the severity of involvement tend to change and are not consistent. Many patients also deal with persistent symptoms in between these acute episodes.
Fortunately, with better management of RP-related complications, survival rates have significantly improved. For instance, in 1986, the 10-year survival rate was only 55%, but by 1998, it had risen to an impressive 94% at the end of 8 years. The primary causes of mortality in these individuals include lower respiratory tract infections, airway collapse, cardiac issues like valvular disease, and advanced systemic vasculitis.[4]
References[edit | edit source]
- ↑ GERGELY P. Relapsing polychondritis. Best Practice & Research Clinical Rheumatology. 2004 Oct;18(5):723–38
- ↑ 2.0 2.1 Kingdon J, Roscamp J, Sangle S, D’Cruz D. Relapsing polychondritis: a clinical review for rheumatologists. Rheumatology. 2017 Nov 6;57(9):1525–32.
- ↑ Puéchal X, Terrier B, Mouthon L, Costedoat-Chalumeau N, Guillevin L, Le Jeunne C. Relapsing polychondritis. Joint Bone Spine [Internet]. 2014 Mar 1 [cited 2020 Oct 30];81(2):118–24. Available from: https://pubmed.ncbi.nlm.nih.gov/24556284/
- ↑ Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clinical Rheumatology. 2013 Jul 26;32(11):1575–83.
