MRI for Screening and Monitoring Scleroderma ILD

Prior treatment

• Any major surgical procedure within 90 days prior to study enrollment or planned surgical procedure during the study period.
• Concomitant medical disorder, condition, or history, that in the opinion of the Investigator would impair the subject's ability to participate in or complete the requirements of the study
• Significant Pulmonary Arterial Hypertension (PAH) defined by any of the following:
  • Previous clinical or echocardiographic evidence of significant right heart failure
  • History of right heart catheterization showing a cardiac index ≤ 2 l/min/m²
  • PAH requiring parenteral therapy with epoprostenol/treprostinil
• Any major surgical procedure within 90 days prior to study enrollment or planned surgical procedure during the study period.
• Concomitant medical disorder, condition, or history, that in the opinion of the Investigator would impair the subject's ability to participate in or complete the requirements of the study
• Subject has been on therapy for SSc-ILD for \>30 days prior to baseline MRI.

Other illness

• Other medical or psychiatric condition which, in the opinion of the Investigator, would place the subject at increased risk or would preclude obtaining voluntary consent or would confound the objectives of the study
  • Arm 2 (Hyperpolarized 129Xe MRI in Patients with Scleroderma ILD)
• Other medical or psychiatric condition which, in the opinion of the Investigator, would place the subject at increased risk or would preclude obtaining voluntary consent or would confound the objectives of the study

Other

• Subject unable to undergo MRI based on MRI safety screening
• Pregnant or breastfeeding female subjects
• Prisoners or incarcerated individuals
• Any in-patient hospitalization (defined as greater than 23 hours) within 30 days of study enrollment
• Subject unable to undergo MRI based on MRI safety screening
• Diagnosis of IPF based on ATS/ERS/JRS/ALAT 2018 Guidelines
• Primary obstructive airway physiology (pre-bronchodilator FEV1/FVC \< 0.7 at Baseline).
• Pregnant or breastfeeding female subjects
• Prisoners or incarcerated individuals
• Any in-patient hospitalization (defined as greater than 23 hours) within 30 days of study enrollment
• Pregnancy (Visit 1 or Visit 2) or intention to become pregnant (Visit 2 only)

• Age ≥ 18 years of age
• Subject has clinical diagnosis of scleroderma.
• Chest CT scan within 1 month prior to screening or Chest CT scan will be completed within 1 month post study enrollment.
• Ability to adhere to the study visit schedule, adhere, and comply with all protocol requirements.
• Ability to understand and provide written informed consent.
• Age ≥ 18 years of age
• Subject clinically diagnosed with SSc-ILD.
• Subject initiating background SSc-ILD therapy within ±30 days of visit 1. (i.e. patient cannot have begun therapy for SSc-ILD more than 30 days prior to visit 1, or must be clinically scheduled to initiate therapy within 30 days after visit 1).
• FVC % Predicted ≥45% pre-bronchodilator within 30 days prior to screening or at baseline.
• DLCO % Predicted ≥30% within 30 days prior to screening or at baseline.
• Oxygen saturation \>87% on room air or with supplemental oxygen
• Ability to adhere to the study visit schedule, adhere, and comply with all protocol requirements.
• Ability to understand and provide written informed consent.

Criteria the registry did not label

• Arm 1 (UTE MRI in patients with Scleroderma)