Chapelon-Abric Catherine, Saadoun David, Marie Isabelle, Comarmond Cloé, Desbois Anne Claire, Domont Fanny, Savey Léa, Cacoub Patrice
Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France.
Sorbonne Universités, UPMC Univ Paris 06, UMR 7211 and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris, France.
Int J Rheum Dis. 2018 Mar;21(3):740-745. doi: 10.1111/1756-185X.13137. Epub 2017 Aug 8.
To describe the features of exceptional coexisting Takayasu arteritis (TA) and sarcoidosis, two conditions of unknown cause associated with a common immunologic pattern.
We report seven cases of concomitant sarcoidosis-Takayasu or Takayasu-like vasculitis, observed in two referral centers between 1995 and 2015.
All patients were female. The mean age at sarcoidosis diagnosis and TA diagnosis was 36 and 37 years, respectively. Sarcoidosis occurred in 86% of cases before or together with TA. Sarcoidosis always had a classic expression except for one renal localization. Sarcoidosis was not severe and mostly non-treated (86%). In all cases of TA, supra-aortic arteries were involved; in only two TA cases a more diffuse inflammatory arterial involvement was noted. In one case, Takayasu arteritis occurred despite immunosuppressive therapy given for sarcoidosis. All patients received for TA a treatment with corticosteroids associated with methotrexate (four cases), infliximab (one case) or tocilizumab (one case). After a mean follow-up of 89 months, TA always improved and no death was observed.
TA stands as pathology associated with sarcoidosis. TA occurred in three cases among 50. When sarcoidosis preceded TA, a recovery of sarcoidosis was achieved mostly without treatment. TA is a prognostic and therapeutic factor. Immunosuppressive treatment, including steroids, led to a good prognosis for TA as well as for sarcoidosis.
描述罕见的同时存在的大动脉炎（TA）和结节病的特征，这两种病因不明的疾病具有共同的免疫模式。
我们报告了1995年至2015年间在两个转诊中心观察到的7例结节病合并TA或TA样血管炎的病例。
所有患者均为女性。结节病诊断和TA诊断时的平均年龄分别为36岁和37岁。86%的病例中结节病发生在TA之前或与之同时发生。除1例肾脏定位外，结节病均有典型表现。结节病病情不严重，多数未接受治疗（86%）。在所有TA病例中，主动脉弓上动脉均受累；仅2例TA病例观察到更广泛的炎症性动脉受累。1例患者尽管因结节病接受了免疫抑制治疗仍发生了大动脉炎。所有TA患者均接受了皮质类固醇联合甲氨蝶呤（4例）、英夫利昔单抗（1例）或托珠单抗（1例）的治疗。平均随访89个月后，TA均有改善，未观察到死亡病例。
TA是与结节病相关的疾病。在50例患者中有3例发生了TA。当结节病先于TA出现时，多数结节病未经治疗即得到缓解。TA是一个预后和治疗因素。包括类固醇在内的免疫抑制治疗对TA和结节病均有良好的预后。
