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[Relapsing polychondritis: What's new in 2017?].

作者信息

Dion J, Leroux G, Mouthon L, Piette J-C, Costedoat-Chalumeau N

机构信息

Service de médecine interne, Centre de référence maladies auto-immunes et systémiques rares d'Île-de-France, hôpital Cochin, université Paris Descartes, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.

Département de médecine interne et d'immunologie clinique, Centre de référence maladies auto-immunes et systémiques rares, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.

出版信息

Rev Med Interne. 2018 Jun;39(6):400-407. doi: 10.1016/j.revmed.2017.07.008. Epub 2017 Sep 8.

DOI:10.1016/j.revmed.2017.07.008
PMID:28890262
Abstract

Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on pathophysiology are scarce and suggest an autoimmune mechanism. Recently, the possibility of dividing patients with RP into three distinct clinical phenotypes has been suggested: the hematological form representing less than 10% of patients, essentially older men with associated myelodysplasia and poor prognosis, the respiratory form representing about 25% of patients with predominant tracheobronchial involvement, and the mild and most frequent form, representing 65% of patients, with a good prognosis. Recent data on survival shows an improvement of overall prognosis compared to historical series. Reported poor prognosis factors are male gender, associated haemopathies and cardiac involvement. Few recent series suggest an interest for positron emission tomography for the diagnosis and the follow-up of treatment. Due to the lack of randomized therapeutic trial, treatment remains empirical and is mainly based on oral corticosteroids sometimes associated with immunosuppressive agents. The use of biologic agents has recently been reported in small retrospective series with different outcome. Finally, some selected patients with mild and occasional peripheral chondritis might justify a treatment with colchicine or a therapeutic abstention with occasional short-term corticosteroids therapy.

摘要

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引用本文的文献

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Unveiling the clinical spectrum of relapsing polychondritis: insights into its pathogenesis, novel monogenic causes, and therapeutic strategies.揭示复发性多软骨炎的临床谱:深入了解其发病机制、新的单基因病因和治疗策略。
Adv Rheumatol. 2024 Apr 16;64(1):29. doi: 10.1186/s42358-024-00365-z.
2
Relapsing Polychondritis: An Updated Review.复发性多软骨炎:最新综述
Biomedicines. 2018 Aug 2;6(3):84. doi: 10.3390/biomedicines6030084.