iloprost and Scleroderma--Limited

The aim of this study was to evaluate pulmonary function in patients suffering from scleroderma treated for 3 years with cyclic iloprost infusions.. Thirty patients affected by scleroderma (five men and 25 women, mean age 49.3 years, mean disease duration 8 years, 19 with limited and 11 with diffuse disease patterns) were treated for 5 consecutive days every 4 months for 3 years with iloprost. Pulmonary function was evaluated at baseline and after 3 years. At the end of the trial, color Doppler echocardiography was performed in 26 of the patients.. Spirometric parameters did not significantly change after 3 years of therapy when expressed as percentage of the predicted normal value. Diffusing lung capacity for carbon monoxide decreased at the limit of statistical significance. No patients developed clinical pictures suggestive of severe pulmonary arterial hypertension, which was confirmed in 26 subjects by evaluation of estimated pulmonary artery systolic pressure.. This long-term study showed that lung function remained substantially stable after 3 years of treatment with cyclic iloprost infusions. No cases of severe pulmonary hypertension were observed.

Topics: Adult; Echocardiography, Doppler, Color; Female; Humans; Iloprost; Infusions, Intravenous; Lung; Male; Middle Aged; Platelet Aggregation Inhibitors; Prospective Studies; Respiratory Function Tests; Scleroderma, Limited; Scleroderma, Systemic; Treatment Outcome; Vasodilator Agents

The pathophysiology of both limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous SSc (dcSSc), representing two subtypes of an autoimmune disease of the connective tissue, is still enigmatic. Life-limiting, progressive fibrotic changes as a consequence of vasculopathy and autoimmunity are characteristic in varying extent for lcSSc and dcSSc. Previously, an increased IL-33 serum concentration in early phase SSc patients and an elevated tissue expression of its receptor, ST2L, on endothelial cells (EC) were described. While suggested as a biomarker for fibrotic diseases, for example liver fibrosis, the role of soluble ST2 (sST2) in the pathological processes and its contribution to vascular fibrosis in SSc has not been investigated. Here, we showed that sST2 is elevated in late phase limited cutaneous SSc (lcSSc) as compared to patients with shorter disease duration or with the diffuse subtype of SSc. We demonstrated that sST2, not IL-33, is significantly increased in serum of lcSSc patients with disease duration over 9 years. Soluble ST2 was not elevated in healthy controls or in SSc patients with early skin involvement or disease duration shorter than 9 years. Furthermore, we observed that sST2 serum levels were lowered by iloprost (prostacyclin) treatment. After 5 days of iloprost infusion, sST2 serum levels fell in 6 of 7 patients. Therefore, we not only like to propose sST2 as a biomarker for progressive vascular fibrosis, but moreover, suggest that the involvement of sST2 in the pathogenesis of lcSSc may be exploited therapeutically.

Topics: Adult; Aged; Autoimmune Diseases; Biomarkers; Disease Progression; Endothelial Cells; Female; Humans; Iloprost; Interleukin-33; Interleukins; Male; Middle Aged; Receptors, Somatostatin; Scleroderma, Diffuse; Scleroderma, Limited; Vasodilator Agents

Digital ulcers in systemic sclerosis are painful ischemic necrotic lesions of the acra. Optimal treatment consists of conventional wound management and medication: iloprost infusions promote primary healing of the ulcers, while the dual endothelin receptor antagonist bosentan is used for secondary prophylaxis of new ulcers. The described case illustrates the essential interdisciplinary collaboration for optimal management of these patients.

Topics: Angiography; Antihypertensive Agents; Arm; Bosentan; Cooperative Behavior; CREST Syndrome; Endothelin Receptor Antagonists; Fatal Outcome; Fingers; Follow-Up Studies; Hand Dermatoses; Humans; Iloprost; Interdisciplinary Communication; Leg; Male; Middle Aged; Scleroderma, Limited; Skin Ulcer; Sulfonamides; Vasodilator Agents