Improvement of lymphangioleiomyomatosis following successful tofacitinib treatment for refractory synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome

作     者：Shuang Liu Chen Li Ming-Wei Tang Wen-Shuai Xu Ke-Qi Chen Xin Sui Xin-Lun Tian Kai-Feng Xu Liu Shuang;Li Chen;Tang Ming-Wei;Xu Wen-Shuai;Chen Ke-Qi;Sui Xin;Tian Xin-Lun;Xu Kai-Feng

作者机构：School of Clinical MedicineChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China Department of Traditional Chinese MedicineChinese Academy of Medical Sciences&Peking Union Medical College HospitalBeijing 100730China Department of Respiratory and Critical Care MedicineChinese Academy of Medical Sciences&Peking Union Medical College HospitalBeijing 100730China Department of RadiologyChinese Academy of Medical Sciences&Peking Union Medical College HospitalBeijing 100730China 

出 版 物：《Chinese Medical Journal》 (中华医学杂志（英文版）)

年 卷 期：2019年第132卷第19期

页      面：2378-2379页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：drugs lung treatment 

摘      要：To the Editor:Lymphangioleiomyomatosis(LAM)is a rare multi-systemic disease that predominantly affects women and is associated with cystic lung destruction,chylous fluid accumulation,and abdominal tumors.[1]The lung function of LAM patients declines at two to four or more times faster rates than the typical age-related decline.Although the mammalian target of rapamycin(mTOR)inhibitor has shown certain benefits for patients with LAM,treatment options remain limited.[2]Synovitis,acne,pustulosis,hyperostosis,and osteitis(SAPHO)syndrome is a spectrum of heterogeneous diseases characterized by osteoarticular and dermatological manifestations.Nonsteroidal anti-inflammatory drugs(NSAIDs),disease-modifying anti-rheumatic drugs,bisphosphonates,and intra-articular steroids are frequently prescribed but are often insufficient to control disease progression.