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

Open to people ages 18-80

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:

1. 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
2. Stable oral therapy for PAH for at least 30 days (monotherapy or combination)
3. New York Heart Association (NYHA) Class I-III Heart Failure

1. Have a diagnosis of systemic sclerosis sine scleroderma;
2. Be less than 18 years of age or greater than or equal to 80 years of age;
3. Be pregnant, nursing, or planning to become pregnant;
4. Current or planned treatment with prostanoid therapy;
5. Current or planned treatment with pirfenidone;
6. Use of rituximab in the last 3 months;
7. Use of mycophenolic acid (Myfortic, CellCept) at a stable dose for less than 3 months;
8. Current or planned corticosteroid therapy greater than 15mg per day of prednisone or prednisone equivalent;
9. Significant lung disease, defined as FVC < 50% predicted or DLCO <40% predicted;
10. . Significant kidney disease, defined as Glomerular Filtration Rate (GFR) < 60 ml/min;
11. . Have moderate or severe hepatic impairment;
12. . Contraindication to MRI (e.g., implanted magnetic material, claustrophobia);
13. . Known hypersensitivity to gadolinium;
14. . Any cause of pulmonary hypertension other than World Health Organization (WHO) Group I associated with SSc;
15. . Use of aspirin > 81 mg per day in the last two weeks;
16. . Use of warfarin, heparin or other anticoagulants in the last 30 days;
17. . Recent (within 6 weeks) myocardial infarction or persistent atrial arrhythmias;
18. . Have a history of allergy or hypersensitivity to ifetroban;
19. . Have taken investigational drugs within 30 days before study treatment administration;
20. . 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;
21. . Be otherwise unsuitable for the study, in the opinion of the investigator.