An Open Label Pilot Study to Assess the Safety and Efficacy of Inhaled Iloprost in Adults with Pulmonary Hypertension Secondary to Interstitial Lung Disease in Systemic Sclerosis.

Study sponsor: Actelion Pharmaceuticals US, Inc.

Coordinating center: University of Michigan

Design:
Open Label study of Inhaled Iloprost for 10-12 patients. Patients will come to the General Clinical Research Center at University of Michigan for a screening visit followed by 4 monthly study visits over a total period of 16 weeks. During the first study visit, patients will be instructed in using the inhalation device by trained clinical personnel. Inhaled Iloprost should be taken 6-9 times a day for the duration of the study. To assess safety of the inhaled Iloprost, patients will undergo the following procedures at each study visit: medical exam, current medication review, review of adverse events (AEs), vitals signs and oxygen saturation (by forehead probe). During the first and last visit, subjects will also have blood samples drawn. During the study visits, patients will undergo a 6 minute walk test (done at each of the 5 visits) and pulmonary function tests (done at the beginning and at the end of the study). They will also be asked to complete questionnaires assessing shortness of breath (Borg and UCSD) and overall health (SHAQ-DI, 20 questions). As part of the study, subjects will answer one question regarding Raynaud’s phenomenon at each study visit and they will undergo assessment of digital ulcers.

Inclusion criteria:
Patients with a diagnosis of SSc by ACR criteria with moderate to severe ILD by HRCT or PFT criteria and PH by RHC utilizing ACCP criteria. 6MWT moderately impaired (150-450m)

Completion: December 2008

Date of expected analysis: End of study.

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