Introduction: The ILD-PRO Registry is a multicenter US registry of patients with progressive fibrosing ILDs other than IPF.

Aim: To assess the demographic and clinical characteristics of patients enrolled into the ILD-PRO Registry.

Methods: Patients enrolled in the ILD-PRO Registry had a non-IPF ILD diagnosed or confirmed at the enrolling center, reticular abnormality and traction bronchiectasis on HRCT and met criteria for ILD progression within the prior 24 months. Descriptive statistics were calculated based on the number of patients with available data.    

Results: Of the first 491 patients enrolled, the majority were white (75.4%) and female (60.6%); 47.4% had history of smoking. Documented ILDs were CTD-ILDs (47.2%), hypersensitivity pneumonitis (17.5%), idiopathic non-specific interstitial pneumonia (9.1%), interstitial pneumonia with autoimmune features (8.9%), unclassifiable ILD (7.6%) and other ILDs (9.7%). Median (Q1, Q3) time from ILD diagnosis to enrollment was 2.0 (1.0, 4.1) years. About half (51.3%) of patients had experienced a relative decline in FVC % predicted ?10% within the prior 24 months. At enrollment, median (Q1, Q3) FVC was 62.2 (49.4, 72.4) % predicted and DLco was 39.2 (30.2, 49.2) % predicted. The most common comorbidities were gastroesophageal reflux disease (61.1%) and sleep apnea (29.6%). Overall, 64.5% of patients were receiving immunosuppressive or cytotoxic therapy, 53.2% oral steroids, 19.8% nintedanib and 3.6% pirfenidone.

Conclusions: Patients enrolled into the ILD-PRO Registry had a variety of ILD diagnoses and marked impairment in lung function. The majority of patients were taking immunosuppressive therapy.