Rehabilitation of Idiopathic Pulmonary Fibrosis (IPF) Patients

Inclusion Criteria:

• Clinical presentation consistent with IPF with onset between three months and 48 months prior to screening.
• Diagnosis made by high-resolution computed tomographic scan showing highly probable IPF.
• Absence of severe pulmonary hypertension (i.e., PAsys must be less than 55 mm Hg, based on echocardiography) and absence of decompensated right heart failure (NYHA class I or II acceptable).
• Age 40 through 80, inclusive.
• Abnormal pulmonary function tests (FVC 40-90% predicted or DLCO 30-90% predicted or impaired gas exchange with rest or exercise).
• Six-minute walk distance 150 m and 500 m.
• Worsening as demonstrated by any one of the following within the past year: > 10% decrease in percent predicted forced vital capacity or worsening dyspnea at rest or upon exertion, based on history.
• Ability to understand and sign a written informed consent form and comply with the requirements of the study.
• Absence of clinical features suggesting infection, neoplasm, sarcoidosis or collagen-vascular disease.

Exclusion Criteria:

• Echocardiographic evidence of severe pulmonary hypertension (PAsys>55 mm Hg, based on echocardiography or TR velocity 3.2 m/sec).
• Severe heart failure (NYHA class III or IV or LVEF < 45%).
• Six-minute walk distance < 150 m or > 500 m.
• FEV1/FVC ratio < 0.7 at screening (post-bronchodilator).
• Residual volume > 100% predicted.
• Any condition other than IPF likely to result in the death of the participant within the next two years.
• History of unstable or deteriorating cardiac or neurologic disease.
• Pregnancy or lactation. Patients who are: (a) pregnant or (b) breast feeding are excluded from the study.
• Current treatment with corticosteroids (either oral or inhaled), Cytoxan, azathioprine, colchicine, pirfenidone, anti-tumor necrosis factor therapy or endothelin receptor blockers. Prior treatment is permitted, but at least four weeks of treatment washout prior to inclusion in this study are required.
• Investigational therapy for any indication within 28 days prior to enrollment.
• Degenerative arthritis, cerebrovascular accident or other limitation to mobility preventing completion of the 6-minute walk test.
• Oxygen saturation on room air <80% at rest.