Clinical Research Directory

Inclusion Criteria: * We will include all patients who are over 18 years of age with a physician-diagnosed ILD of one of the below subtypes based on multidisciplinary consensus 1. Chronic hypersensitivity pneumonitis 2. Autoimmune interstitial lung disease (including rheumatoid arthritis-ILD, mixed connective tissue disorder related ILD, myositis related ILD, scleroderma related ILD, and idiopathic pneumonia with autoimmune features) 3. Idiopathic NSIP 4. Unclassifiable idiopathic interstitial pneumonia * Fibrotic lung disease affecting more than 10% of lung volume on high-resolution CT, per Duke radiology review * Evidence of any of the following criteria for progression of ILD within the 24 months before screening: 1. Relative decline in FVC % predicted of at least 10% 2. Relative decline in FVC % predicted ≥ 5% - \< 10 combined with either increasing extent of fibrotic changes on HRCT or worsening of respiratory symptoms 3. Worsening respiratory symptoms and increased extent of fibrosis on HRCT * Willing and able to give informed consent and adhere to visit/protocol schedules * Immunosuppressive medication, including azathioprine, cyclosporine, mycophenolate mofetil, rituximab, cyclophosphamide, or oral glucocorticoids are permitted at the discretion of the treating physician Exclusion Criteria: * Subject is less than 18 years of age * Prior treatment with nintedanib or pirfenidone * Subject is pregnant or lactating * Prior investigational drug use within 28 days * MRI is contraindicated based on responses to MRI screening questionnaire * Respiratory illness of a bacterial or viral etiology within 30 days of MRI * Acute exacerbation within 30 days of MRI, defined by acute increases in oxygen requirement, bilateral alveolar filling opacities on imaging, and the need for antibiotics and/or systemic steroids * Subject does not fit into 129Xe vest coil used for MRI * Subject with ventricular cardiac arrhythmia in the past 30 days. * Subject has history of cardiac arrest within the last year * Subject deemed unlikely to be able to comply with instructions during imaging * Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements