Oral Ifetroban to Treat Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension

Prior treatment

• Current or planned treatment with prostanoid therapy;
• Current or planned treatment with pirfenidone;
• Current or planned corticosteroid therapy greater than 15mg per day of prednisone or prednisone equivalent;
• Have taken investigational drugs within 30 days before study treatment administration;

Other illness

• Significant lung disease, defined as FVC \< 50% predicted or DLCO \<40% predicted;
• Have moderate or severe hepatic impairment;
• Known hypersensitivity to gadolinium;
• Any cause of pulmonary hypertension other than World Health Organization (WHO) Group I associated with SSc;
• Recent (within 6 weeks) myocardial infarction or persistent atrial arrhythmias;
• Have a history of allergy or hypersensitivity to ifetroban;

Lab values

• Significant kidney disease, defined as Glomerular Filtration Rate (GFR) \< 60 ml/min;

Age

• Be less than 18 years of age or greater than or equal to 80 years of age;

Other

• Have a diagnosis of systemic sclerosis sine scleroderma;
• Be pregnant, nursing, or planning to become pregnant;
• Use of rituximab in the last 3 months;
• Use of mycophenolic acid (Myfortic, CellCept) at a stable dose for less than 3 months;
• Contraindication to MRI (e.g., implanted magnetic material, claustrophobia);
• Use of aspirin \> 81 mg per day in the last two weeks;
• Use of warfarin, heparin or other anticoagulants in the last 30 days;
• Inability to understand the requirements of the study, inability to understand spoken English and abide by the study restrictions and to return for the required treatments and assessments;
• Be otherwise unsuitable for the study, in the opinion of the investigator.

• Diffuse Cutaneous Criterion:
• 1\. Systematic Sclerosis (SSc), as defined using the 2013 American College of Rheumatology/ European Union League Against Rheumatism Classification Criteria and dcSSc within 7 years following initial diagnosis as defined by the onset of the first non-Raynaud symptom.
• SSc-PAH Criteria:
• Adults fulfilling the 2013 American College of Rheumatology/ European Union League Against Rheumatism Classification Criteria with confirmed SSc-PAH (limited or dcSSc) confirmed via previous cardiac catheterization
• Stable oral therapy for PAH for at least 30 days (monotherapy or combination)
• New York Heart Association (NYHA) Class I-III Heart Failure