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

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.

Study Overview

• Status: Recruiting
• Conditions: Systemic Sclerosis; Interstitial Lung Disease

• Study Type: Observational
• Enrollment (Estimated): 20

Contacts and Locations

• Study Contact: Name: Paul Decker, MD; Phone Number: +33383157240; Email: p.decker@chru-nancy.fr

Study Locations

• France
  • Grand Est
    • Vandœuvre-lès-Nancy, Grand Est, France, 54500
      • Recruiting
      • CHU Nancy
      • Contact: Paul DECKER, MD; Phone Number: +3383157240; Email: p.decker@chru-nancy.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients with systemic sclerosis-interstitial lung disease

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
SSc-ILD patients with JAK inhibitors

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
relative change in FVC after 12 months of JAK inhibitor	relative change in %predicted FVC after 12 months of JAK inhibitor	at JAK inhibitor initiation (J0) and 12 months after JAK inhibitor initiation

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
relative change in DLCO after 12 months of JAK inhibitor	relative change in %predicted DLCO after 12 months of JAK inhibitor	at JAK inhibitor initiation (J0) and 12 months after JAK inhibitor initiation
relative change in skin fibrosis after 12 months of JAK inhibitor	relative change in modified Rodnan skin score after 12 months of JAK inhibitor	at JAK inhibitor initiation (J0) and 12 months after JAK inhibitor initiation

Collaborators and Investigators

• Sponsor: Central Hospital, Nancy, France

Study record dates

Study Major Dates

• Study Start (Actual): January 19, 2022
• Primary Completion (Estimated): July 4, 2024
• Study Completion (Estimated): July 4, 2024

Study Registration Dates

• First Submitted: December 15, 2021
• First Submitted That Met QC Criteria: December 15, 2021
• First Posted (Actual): January 4, 2022

Study Record Updates

• Last Update Posted (Actual): August 3, 2023
• Last Update Submitted That Met QC Criteria: August 2, 2023
• Last Verified: August 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Skin Diseases; Respiratory Tract Diseases; Connective Tissue Diseases; Sclerosis; Lung Diseases; Scleroderma, Systemic; Scleroderma, Diffuse; Lung Diseases, Interstitial
• Other Study ID Numbers: 2021PI233

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No