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

Status

Withdrawn

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT05177471

Acronym

SCLEROJAKI

Enrollment

Registered

2022-01-04

Start date

2022-01-19

Completion date

2024-08-26

Last updated

2024-08-27

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Interstitial Lung Disease, Systemic Sclerosis

Brief summary

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.

Interventions

None listed

Study design

Observational model

COHORT

Time perspective

RETROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

18 Years to No maximum

Healthy volunteers

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

Design outcomes

Primary

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

Secondary

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

Countries

France

Outcome results