title: Low-dose imatinib in the treatment of severe systemic sclerosis: a case series of six Chinese patients and literature review.
abstract: Systemic sclerosis (SSc) is a progressive fibrotic disorder with no legitimate effective treatment. Several clinical trials had investigated imatinib mesylate with a target dose of 400∼600 mg/day on SSc, and the efficacy is controversial with a generally poor tolerability. We herein reported six female Chinese patients with SSc administered with low-dose imatinib (200 mg/day) for a median of 23 months (10∼30 months). Patients displayed a decreased modified Rodman skin scores (mRSS) by a mean of 6.29 points after 6 months of treatment. Three patients who completed 2 years of treatment achieved a reduction of mRSS by 8, 18, and 30.5 points, respectively. Pulmonary function was improved or stabilized in two patients with interstitial lung disease. Severe gastrointestinal involvement in one patient was attenuated in terms of discontinuation of total parenteral nutrition and restoration of the serum albumin level. Imatinib was well-tolerated in general, although there were two severe adverse events: a bone fracture and a cerebral hemorrhage in two individuals. Both the adverse events were probably not directly related to imatinib and were recovered uneventfully. Our limited data, along with the review of the literature, suggested that low-dose imatinib might be effective and better tolerated in severe SSc that deserves further study.
authors.affiliation: Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, 145 Shandong C Rd, Shanghai 200001, China.
authors.forename: Li
authors.initials: L
authors.lastname: Guo
authors.forename: Xiao-xiang
authors.initials: XX
authors.lastname: Chen
authors.forename: Yue-ying
authors.initials: YY
authors.lastname: Gu
authors.forename: He-jian
authors.initials: HJ
authors.lastname: Zou
authors.forename: Shuang
authors.initials: S
authors.lastname: Ye
mesh_terms: D000293:Adolescent; D000328:Adult; D001327:Autoimmune Diseases / Q000378:metabolism; D001549:Benzamides; D002681:China; D002986:Clinical Trials as Topic; D005260:Female; D005355:Fibrosis; D006801:Humans; D000068877:Imatinib Mesylate; D008168:Lung / Q000502:physiology; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D012595:Scleroderma, Systemic / Q000188:drug therapy* / Q000208:ethnology*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate