Double Immunosuppression With or Without Anti-fibrotic in Scleroderma ILD

University of Patras

Status: Enrolling

Conditions: SSc-Systemic Sclerosis

Treatments: Drug: MMF Immunosuppression; Drug: Antifibrotic drugs (nidanib or pirfenidone)

Study type: Observational

Funder types: Other

Identifiers

NCT07491523

University of Patra

Details and patient eligibility

About

Patients with ssc-ild receiving double immunosuppression with or without anti fibrotic tratment

Full description

Systemic sclerosis (scleroderma) is a rare systemic autoimmune disease. It primarily affects the skin, but it can also involve other vital organs and systems, and is characterized by fibrosis, vascular abnormalities, and the production of autoantibodies. Its main pathological feature is the excessive production and deposition of collagen in the skin and other organs.

Mortality in scleroderma varies depending on the type and severity of the disease, particularly on whether vital organs are involved. Patients with limited cutaneous scleroderma generally have a better prognosis and life expectancy, whereas those with diffuse scleroderma face a higher risk of death from disease-related complications, mainly due to lung involvement.

Pulmonary involvement in scleroderma is common, either affecting the pulmonary blood vessels (pulmonary hypertension) or the supporting structure (interstitium) of the lungs, leading to interstitial fibrosis.

Objective:

The aim of the study is to compare patients receiving only dual immunosuppressive therapy (control group) with patients receiving dual immunosuppressive therapy combined with antifibrotic treatment (e.g., nintedanib).

Methods:

This will be a retrospective study including patients with scleroderma and diffuse pulmonary fibrosis who are followed at the Rheumatology Department of the University General Hospital of Patras and are receiving the two proposed treatment regimens. Because fibrosis and its progression are slow processes, we propose a comparative analysis of outcomes in both groups at 2 years from treatment initiation.

Inclusion criteria:

Presence of pulmonary fibrosis (documented by chest HRCT), regardless of whether pulmonary function tests show a restrictive pattern or not.

Availability of pulmonary function tests for each patient at the following time points:

0 months (baseline) 6 months 12 months Patients (controls) should be receiving either dual therapy (MMF + RTX) or triple therapy (MMF + RTX + nintedanib).

Analysis:

Statistical analysis will be performed using the unpaired two-tailed Student's t-test.

Expected results / originality / contribution to science:

To date, no similar study exists in the international literature. Since interstitial lung disease is the leading cause of death in patients with scleroderma, the results of this study may contribute to improved management. We hypothesize that the combination of dual immunosuppressive therapy and antifibrotic treatment will be superior to dual immunosuppressive therapy alone.

Enrollment: 35 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion and exclusion criteria

Inclusion Criteria:ild-ssc with double immunosuppression

-

Exclusion Criteria:

• ssc without ild

Trial design

35 participants in 2 patient groups

Patient with ssc-ild with a double immunosuppression treatment and with anti fibrotic

Description:

Comparison of the two groups

Treatment:

Drug: Antifibrotic drugs (nidanib or pirfenidone)

Drug: MMF Immunosuppression

Patient with ssc-ild with a double immunosuppression treatment only

Description:

Patients with rituximab and mmf

Treatment:

Drug: MMF Immunosuppression