title: Efficacy of low-dose imatinib mesylate for cutaneous involvement in systemic sclerosis: a preliminary report of three cases.
abstract: In this pilot study, the effect of low-dose imatinib mesylate (100 mg/day) on cutaneous involvement in patients with systemic sclerosis (SSc) was analyzed. Three patients with SSc were treated with 100 mg/day of imatinib mesylate for 6 months because of pulmonary arterial hypertension refractory to conventional treatments, including beraprost, bosentan, sildenafil, and epoprostenol. Changes in cutaneous involvement were evaluated at 1, 3, and 6 months. During the treatment, the total skin score gradually improved in all of the patients. Contracture of phalanges was attenuated in two patients, one of whom also experienced the partial restoration of large-joint mobility. Nailfold bleeding, initially seen in two patients, was gradually attenuated and had completely disappeared at 6 months. In all patients, Raynaud's phenomenon was attenuated at around 3 months and had completely disappeared at 6 months. Although transient renal dysfunction was observed in one patient, none of the patients experienced common adverse effects of imatinib, such as edema, nausea, rash, and musculoskeletal pain. These clinical data indicate the tolerability and efficacy of low-dose imatinib in SSc, especially against cutaneous vascular involvement, including Raynaud's phenomenon and nailfold bleeding.
authors.affiliation: Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
authors.forename: Zenshiro
authors.initials: Z
authors.lastname: Tamaki
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Asano
authors.forename: Masaru
authors.initials: M
authors.lastname: Hatano
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yao
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Kawashima
authors.forename: Manabu
authors.initials: M
authors.lastname: Tomita
authors.forename: Koichiro
authors.initials: K
authors.lastname: Kinugawa
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
mesh_terms: D000368:Aged; D001549:Benzamides; D003286:Contracture / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D004305:Dose-Response Relationship, Drug; D005260:Female; D006470:Hemorrhage / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D000068877:Imatinib Mesylate; D008297:Male; D008875:Middle Aged; D009260:Nail Diseases / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D009262:Nails / Q000098:blood supply / Q000473:pathology; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D016059:Range of Motion, Articular; D011928:Raynaud Disease / Q000188:drug therapy / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D012867:Skin / Q000098:blood supply / Q000187:drug effects* / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate