Efficacy and Safety of JAK Inhibitors in Systemic Sclerosis-associated Interstitial Lung Disease

Efficacy and Safety of JAK Inhibitors in Patients With Systemic Sclerosis and Interstitial Lung Disease

Systemic sclerosis (SSc) is a heterogeneous systemic autoimmune disease with distinct prognosis according to patients. In patients with systemic sclerosis, interstitial lung disease (ILD) concerns almost 50 % of patients and represents the main cause of mortality.

Janus kinases (JAK) inhibitors are recent therapies in the field of systemic autoimmune diseases, already approved in patients with rheumatoid arthritis.

Use of JAK inhibitors in systemic sclerosis is based on their anti-inflammatory and anti-fibrotic properties. Several preclinical murine models of systemic sclerosis demonstrated the efficacy of ruxolitinib and tofacitinib on cutaneous and pulmonary fibrosis. Recently, tofacitinib was evaluated in SSc patients in two clinical studies and showed significant improvement on skin fibrosis.

The objective of this study is to evaluate efficacy and safety of JAK inhibitors in SSc patients with ILD.

Inclusion Criteria:

• Patients with systemic sclerosis according to 2013 ACR/EULAR criteria
• Patients with interstitial lung disease affecting at least 10 % of the lungs on HRCT chest, FVC of at least 40 % of the predicted value and DLCO between 30 % and 90 % of the predicted value
• Use of JAK inhibitors

Exclusion Criteria:

• Patients with an alternative diagnosis of SSc-associated ILD (silicosis, sarcoidosis, lung cancer or other significant lung abnormalities)
• Patients with pulmonary arterial hypertension defined on right heart catheterization