Abstract
This work is aimed to study the clinical and prognostic features of relapsing polychondritis (RP) in China. A total of 158 RP cases from 1985 to 2013 in China were included and compared with international case series in terms of clinical features, systemic involvement, differential diagnosis and prognosis. (1) The average age at the onset was 45.3 years old, the average age for initial symptoms was 14.4 months, female/male ratio was 0.7:1 and misdiagnosis rate was 47 %. (2) The incidence of arthritis was lower than that in Caucasians. The incidences of auricular chondritis (68 %: 84–95 %), ocular inflammation (44 %: 49–65 %) and renal involvement (3 %: 7–26 %) were lower, and laryngotracheal symptoms (69 %: 31–67 %), skin (46 %: 4–38 %) and neurological involvement (12 %: 2–8 %) were higher during the follow-up period. The proportion of associated autoimmune disease and systemic vasculitis were 5 and 3 %, respectively, similar to that in Japanese (4 and 2 %), but less than that in Caucasians (12–31 and 8–18 %) except the Francès’s study (7 and 3 %). The primary death cause is respiratory failure due to RP, followed by lung infections and cardiovascular events. (3) Juvenile RP (onset ≤18 years) was more severe than adults, similar to results from the Caucasians. However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40–47 %), arthritis (100 %: 71–90 %), cardiovascular (14 %: 3–10 %) and skin involvement (20 %: 10–11 %) than Caucasian juvenile RP. Although sharing most of the clinical features with case series in previous literature, Chinese patients with RP have its unique characteristics.
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References
Foidart JM, Abe S, Martin GR et al (1978) Antibodies to type II collagen in relapsing polychondritis. N Engl J Med 299(22):1203–1207
Buckner JH, Wu JJ, Reife RA, Terato K, Dr Eyre (2000) Autoreactivity against matrilin-1 in a patient with relapsing polychondritis. Arthritis Rheum 43(4):939–943
Suzuki M, Uchida K, Nagano M et al (1983) Case of relapsing polychondritis associated with persistent rib cartilage pain and severe tracheal stenosis–a review of 53 cases in Japan. Nihon Kyobu Shikkan Gakkai zasshi 21(7):665–671
Ananthakrishna R, Goel R, Padhan P, Mathew J, Danda D (2009) Relapsing polychondritis—case series from South India. Clin Rheumatol 28(Suppl 1):S7–10
Sharma A, Bambery P, Wanchu A et al (2007) Relapsing polychondritis in North India: a report of 10 patients. Scand J Rheumatol 36(6):462–465
Kong KO, Vasoo S, Tay NS, Chng HH (2003) Relapsing polychondritis—an oriental case series. Singap Med J 44(4):197–200
Damiani JM, Levine HL (1979) Relapsing polychondritis—report of ten cases. Laryngoscope 89(6 Pt 1):929–946
Mcadam LP, o’Hanlan MA, Bluestone R, Pearson CM (1976) Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Med (Baltim) 55(3):193–215
Michet CJ Jr, Mckenna CH, Luthra HS, Fallon WM (1986) Relapsing polychondritis: survival and predictive role of early disease manifestations. Ann Intern Med 104(1):74–78
Berger R (1998) Polychondritis resulting from intravenous substance abuse. Am J Med 85(3):415–417
Trentham DE, Le CH (1998) Relapsing polychondritis. Ann Intern Med 129(2):114–122
Zeuner M, Straub RH, Rauh G, Albert ED, Scholmerich J, Lang B (1997) Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 24(1):96–101
Chang-Miller A, Okamura M, Torres VE et al (1987) Renal involvement in relapsing polychondritis. Med (Baltim) 66(3):202–217
Frances C, El Rassi R, Laporte JL, Rybojad M, Papo T, Piette JC (2001) Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center. Med (Baltim) 80(3):173–179
Marshall DA, Jackson R, Rae AP, Capell HA (1992) Early aortic valve cusp rupture in relapsing polychondritis. Ann Rheum Dis 51(3):413–415
Sundaram MB, Rajput AH (1983) Nervous system complications of relapsing polychondritis. Neurology 33(4):513–515
Lang-Lazdunski L, Hvass U, Paillole C, Pansard Y, Langlois J (1995) Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis 4(3):227–235
Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN (2001) Active aortitis in relapsing polychondritis. J Clin Pathol 54(11):890–892
Carter JD (2005) Treatment of relapsing polychondritis with a TNF antagonist. J Rheumatol 32(7):1413
Belot A, Duquesne A, Job-Deslandre C et al (2010) Pediatric-onset relapsing polychondritis: case series and systematic review. J Pediatr 156(3):484–489
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Dong-Fang Lin and Wei-Qiang Yang contributed as the co-first author.
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Lin, DF., Yang, WQ., Zhang, PP. et al. Clinical and prognostic characteristics of 158 cases of relapsing polychondritis in China and review of the literature. Rheumatol Int 36, 1003–1009 (2016). https://doi.org/10.1007/s00296-016-3449-8
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DOI: https://doi.org/10.1007/s00296-016-3449-8