title: A case report: The efficacy of pirfenidone in a Chinese patient with progressive systemic sclerosis-associated interstitial lung disease: A CARE-compliant article.
abstract: Systemic sclerosis (SSc)-associated interstitial lung disease (SSc-ILD) has become the leading SSc-related cause of death. Although various types of immunosuppressive therapy have been attempted for patients with SSc-ILD, no curative or effective treatment strategies for SSc-ILD have been developed. Therefore, management of patients with SSc-ILD remains a challenge. Here, we report a Chinese, female, SSc-ILD patient who was negative for Scl-70 and showed an excellent response to pirfenidone without obvious adverse effects. She had been suffered from dry cough and exertional dyspnea for 2 months. The chest computed tomography manifestation was consistent with a pattern of fibrotic nonspecific interstitial pneumonia. The pulmonary function test showed isolated impaired diffusion. After 11 weeks of administration of pirfenidone, the dry cough and dyspnea had disappeared. Both of the lung shadows and the pulmonary diffusion function were improved. Pirfenidone might be an effective option for early SSc-ILD treatment. A well-controlled clinical trial is expected in the future.
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Pathological Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Radiological Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng, Beijing, China.
authors.forename: Hui
authors.initials: H
authors.lastname: Huang
authors.forename: Rui E
authors.initials: RE
authors.lastname: Feng
authors.forename: Shan
authors.initials: S
authors.lastname: Li
authors.forename: Kai
authors.initials: K
authors.lastname: Xu
authors.forename: Ya Lan
authors.initials: YL
authors.lastname: Bi
authors.forename: Zuo Jun
authors.initials: ZJ
authors.lastname: Xu
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000188:drug therapy*; D008875:Middle Aged; D011728:Pyridones / Q000627:therapeutic use*; D012595:Scleroderma, Systemic / Q000150:complications*; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone