Assistance Publique- Hôpitaux de Paris (AP-HP), Hôpital Avicenne, Service de Médecine Interne, Université Paris 13, Sorbonne Paris Cité, Faculté de Médecine SMBH, 125 Route de Stalingrad, 93009, Bobigny Cedex 9, France.
AP-HP, Hôpital Avicenne, Service de Médecine Nucléaire, Université Paris 13, Sorbonne Paris Cité, Faculté de Médecine SMBH, Bobigny, France.
Rheumatol Int. 2017 Nov;37(11):1931-1935. doi: 10.1007/s00296-017-3832-0. Epub 2017 Sep 30.
Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition.
复发性多软骨炎(RP)是一种罕见的全身性炎症性疾病,主要影响耳朵、鼻子和气管支气管树的软骨,但也会影响心血管系统。心血管并发症是 RP 患者死亡的第二大原因。我们报告了一例皮质类固醇耐药性 RP 相关主动脉炎患者,该患者成功接受了托珠单抗(TCZ)治疗。FDG-PET/CT 是诊断主动脉炎和评估生物治疗效果的有用工具。我们进行了系统的文献回顾,证实这是首例皮质类固醇耐药性 RP 相关主动脉炎患者在接受 TCZ 作为一线免疫治疗后迅速和持续缓解的病例。然而,需要进一步的研究来证实 TCZ 在这种危及生命的情况下的有益效果。