摘要
患者男性,51岁。因双眼突出、头痛、视物模糊半年就诊。主要临床特征为球后炎性假瘤及腹膜后纤维化,初诊为肉芽肿性多血管炎(GPA),先后予地塞米松10 mg/d静脉输液5 d,口服泼尼松1 mg·kg^(-1)·d^(-1)1个月后逐渐减量,同时联合口服环磷酰胺100 mg/d治疗后,病情曾一度维持稳定,但诊治过程中发现硬脑膜占位、骨质硬化等无法用GPA解释,经病理活检及基因检查诊断为脂质肉芽肿病(ECD),加用维罗替尼治疗(480 mg,2次/d),目前患者病情稳定,仍在随访中。临床医生应提高对ECD的认识与重视。
One 51 years old man was admitted to the rheumatology department with a history of prominent eyes,headache and blurred vision for half year.The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis.He was initially diagnosed as granulomatosis with polyangiitis.Prednisone and cyclophosphamide were administrated and effective.New mass of dura mater and osteosclerosis presented during follow up.Finally Erdheim Chester disease(ECD)was diagnosed by biopsy and pathological examination.Vemurafenib,a v-raf murine sarcoma viral oncogenes homolog B1(BRAF)inhibitor,480 mg was given twice a day.The patient′s condition is stable and still in follow-up.Although ECD is a rare histiocytosis,clinicians should pay attention to its manifestations and differential diagnoses.
作者
杨云娇
曹欣欣
王凤丹
李烁
李明利
李菁
田新平
曾小峰
Yang Yunjiao;Cao Xinxin;Wang Fengdan;Li Shuo;Li Mingli;Li Jing;Tian Xinping;Zeng Xiaofeng(Department of Rheumatology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,China National Clinical Research Center for Dermatologic and Immunologic Disease,Beijing 100730,China;Department of Hematology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Radiology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)
出处
《中华内科杂志》
CAS
CSCD
北大核心
2021年第4期388-392,共5页
Chinese Journal of Internal Medicine
关键词
脂质肉芽肿病
肉芽肿性多血管炎
球后炎性假瘤
Erdheim-Chester disease
Granulomatosis with polyangiitis
Retrobulbar inflammatory pseudotumor