Vol.22 No.1

Original Article

Efficacy of low-dose imatinib mesylate for cutaneous involvement in systemic sclerosis: a preliminary report of three cases

Authors

Zenshiro Tamaki¹ , Yoshihide Asano¹ , Masaru Hatano² , Atsushi Yao² , Tomohiko Kawashima¹ , Manabu Tomita¹ , Koichiro Kinugawa² , Ryozo Nagai² , Shinichi Sato¹

Received:

21 February 2011

Accepted:

10 May 2011

Published online:

3 June 2011

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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.

Key words

Angiopathy - Fli1 - Imatinib mesylate - Raynaud’s phenomenon - Systemic sclerosis