Long-term treatment of patients affected by systemic sclerosis with cyclosporin A

Abstract

Sir, Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular lesions and widespread fibrosis [1]. The therapy of SSc is still a matter of discussion [2]. Since 1993 we have treated SSc patients with low oral doses of cyclosporin A (CyA) associated or not with vasodilator drugs [3]. The rationale for the use of CyA in SSc lies in the fact that CyA blocks the activation of nuclear factors, such as NFAT and OAP, that are involved in the induction of mRNA transcription for several immune‐activating and proinflammatory cytokines [4]. We have already described the results obtained after 1 yr of therapy with CyA and Iloprost infusion [5]. To verify whether therapeutic results can be maintained with long‐term treatment without side‐effects, we treated nine patients (seven females and two males, mean age 37.5 yr) affected by diffuse SSc (according to the major criteria of the American College of Rheumatology [6]) with CyA at 2.5 mg/kg/day for a period ranging from 3 to 5 yr. No patient used corticosteroids before and during the course of CyA therapy. The clinical analysis was performed by plicometry, oesophageal manometry and 24‐h measurement of pH, spirometry, intrarenal duplex Doppler sonography, echocardiography and nailfold videocapillaroscopy, as described [5]. The results were expressed as scores according to a method of standardization described previously [5, 7]. The t‐test for non‐parametric data was used to detect statistically significant differences between means of scores.