A Study Using a Disease Registry to Observe the Long-term Effects of Nintedanib in People With Scleroderma-related Lung Fibrosis

Time to absolute (% predicted) FVC decline ≥5%

Time frame: up to 10 years

Time to absolute (% predicted) FVC decline ≥10%

Time frame: up to 10 years

Time to relative (ml) FVC decline ≥5%

Time frame: up to 10 years

Time to relative (ml) FVC decline ≥10%

Time frame: up to 10 years

Time to lung transplantation (indicating end-stage ILD)

Time frame: up to 10 years

Time to death

Time frame: up to 10 years

Change from baseline in FVC (% predicted)

Time frame: at baseline, up to 10 years

Change from baseline in FVC (ml)

Time frame: at baseline, up to 10 years

Lung specific Quality of Life (QoL) measured as change from baseline of total, impact, and symptom scores using the Living with Pulmonary Fibrosis (L-PF) questionnaire, measured as change of baseline at years 1, 2, 3,….10

The L-PF patient reported outcome measure (PROM) is a questionnaire designed for patients with fibrosing ILDs and developed with those patients to investigate their level of symptoms and QoL. The L-PF consists of two modules, symptoms and impacts, and five scales: symptoms total, dyspnea, cough, fatigue, impacts total, and Total L-PF score. Items in both modules have response options on a five-option numeric rating score with an anchor of 0 "Not at all" to 4 "Extremely". Overall scores range from 0 to 100, with higher numbers indicating a greater impairment.

Time frame: at baseline, up to 10 years

Time to worsening of Pulmonary Hypertension (PH) in the sub-population of patients with PH at baseline

Worsening of PH is defined as a composite outcome of the following: 1. time to all-cause death; 2. time to non-planned PH-related hospitalization; or 3) time to PH-related deterioration identified by at least one of the following parameters: i. increase in World Health organization functional class, ii. deterioration in exercise testing; or iii. Signs or symptoms of right-sided heart failure

Time frame: up to 10 years

Incidence rate of major bleeding (defined as those requiring intervention or hospitalization) in exposed vs unexposed treatment groups

Time frame: up to 10 years

Final outcome for the major bleeding events (i.e., recovered or fatal) overall

Time frame: up to 10 years

Proportion of patients with major bleeding that had a pre-existing risk for bleeding

Time frame: up to 10 years

Incidence rate of gastrointestinal perforations in exposed vs unexposed treatment groups

Time frame: up to 10 years

Final outcome (i.e., recovered or fatal) for gastrointestinal perforation events overall

Time frame: up to 10 years

Proportion of patients with gastrointestinal perforations that had a pre-existing risk for gastrointestinal perforations

Time frame: up to 10 years

Incidence rate of thromboembolism (arterial or venous) in exposed vs unexposed treatment groups

Time frame: up to 10 years

Final outcome (i.e., recovered or fatal) for thromboembolic events overall

Time frame: up to 10 years

Proportion of patients with thromboembolism that had a pre-existing risk for thromboembolism

Time frame: up to 10 years