MRI for Screening and Monitoring Scleroderma ILD

Arm 1 (UTE MRI in patients with Scleroderma)

Inclusion Criteria:

• 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 completedwithin 1 month post study enrollment.

• Ability to adhere to the study visit schedule, adhere, and comply with all protocolrequirements.

• Ability to understand and provide written informed consent.

Exclusion Criteria:

• 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 ofstudy enrollment

• Any major surgical procedure within 90 days prior to study enrollment or plannedsurgical procedure during the study period.

• Concomitant medical disorder, condition, or history, that in the opinion of theInvestigator would impair the subject's ability to participate in or complete therequirements of the study

• Other medical or psychiatric condition which, in the opinion of the Investigator,would place the subject at increased risk or would preclude obtaining voluntaryconsent or would confound the objectives of the study

Arm 2 (Hyperpolarized 129Xe MRI in Patients with Scleroderma ILD)

Inclusion Criteria:

• 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, ormust be clinically scheduled to initiate therapy within 30 days after visit 1).

• FVC % Predicted ≥45% pre-bronchodilator within 30 days prior to screening or atbaseline.

• 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 protocolrequirements.

• Ability to understand and provide written informed consent.

Exclusion Criteria:

• Subject unable to undergo MRI based on MRI safety screening

• Diagnosis of IPF based on ATS/ERS/JRS/ALAT 2018 Guidelines

• Significant Pulmonary Arterial Hypertension (PAH) defined by any of the following:

1. Previous clinical or echocardiographic evidence of significant right heartfailure

2. History of right heart catheterization showing a cardiac index ≤ 2 l/min/m²

3. PAH requiring parenteral therapy with epoprostenol/treprostinil

• Primary obstructive airway physiology (pre-bronchodilator FEV1/FVC < 0.7 atBaseline).

• Pregnant or breastfeeding female subjects

• Prisoners or incarcerated individuals

• Any in-patient hospitalization (defined as greater than 23 hours) within 30 days ofstudy enrollment

• Any major surgical procedure within 90 days prior to study enrollment or plannedsurgical procedure during the study period.

• Concomitant medical disorder, condition, or history, that in the opinion of theInvestigator would impair the subject's ability to participate in or complete therequirements of the study

• Other medical or psychiatric condition which, in the opinion of the Investigator,would place the subject at increased risk or would preclude obtaining voluntaryconsent or would confound the objectives of the study

• Subject has been on therapy for SSc-ILD for >30 days prior to baseline MRI.

• Pregnancy (Visit 1 or Visit 2) or intention to become pregnant (Visit 2 only)