Treatment and Prevention of Progression of Interstitial Lung Disease in Systemic Sclerosis

• Conditions: Systemic Sclerosis; Interstitial Lung Diseases

• Registration Number: NCT01858259
• Lead Sponsor: Gabriela Riemekasten

Brief Summary

Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and all internal organs. Interstitial lung disease is a frequent morbidity and mortality-driving manifestation in systemic sclerosis.

This observational trial (OT) is part of the collaborative project "DeSScipher", one out of five OTs to decipher the optimal management of systemic sclerosis. Aim of this observational try is to identify:

* The state of clinical practice in Europe for prevention and treatment of interstitial lung disease and its impact on lung function and disease progression

* The potential predictors and confounders for response to therapy

Detailed Description

Patients are routinely evaluated every 3 months over a 12-months period by medical history, physical examination, pulmonary function tests, VAS lung score and SF-36, SHAQ. Also, their medication and possible medication changes will be recorded.

Study Timeline

• Study Start: 2013-05
• Study First Submitted: 2013-05-14
• Study First Submitted QC: 2013-05-16
• Study First Posted: 2013-05-21
• Status Verified: 2014-08
• Last Update Submitted: 2014-08-12
• Last Update Posted: 2014-08-13
• Primary Completion: 2016-06
• Study Completion: 2016-06

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 1372

Inclusion Criteria

• Diagnosis fo SSc according to the ACR/EULAR criteria for adult or the PRES/ACR/EULAR criteria for juvenile SSc patients
• SSc patients with proven ILD (by X-ray or CT scan)
• Treatment with standard dosages according to current practice with (i) cyclophosphamide, (ii) azathioprine, (iii) mycophenolate mofetil, (iv) methotrexate, or (v) no therapy

Exclusion Criterion:

• Patients with previous exposure to silica or asbestos

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Proportion of patients with 10% decline in FVC	1 year	The proportion of patients with ILD progression as defined by a 10% decline in FVC within 1 year of therapy

Secondary Outcome Measures

Name	Time	Method
The need for oxygen support	1 year
The time to a 15% decline in DLCO or a drop <55% of predicted lung function	1 year
The mortality due to lung fibrosis	1 year

Trial Locations

Locations (11)

University of Zurich, Department of Rheumatology; 🇨🇭 Zurich, Switzerland

Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016; 🇫🇷 Paris, France

Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology; 🇩🇪 Bad Nauheim, Germany

University of Florence, Denothe Centre, Division of Rheumatology AOUC, Department of Biomedicine; 🇮🇹 Firenze, Italy

Policlinico, Via Pansini; 🇮🇹 Napoli-Italia, Italy

Felix-Platter Spital, University of Basel; 🇨🇭 Basel, Switzerland

Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie; 🇩🇪 Berlin, Germany

Pecsi Tudomanyegyetem - University of Pecs; 🇭🇺 Pecs, Hungary

Centre for Pediatric Rheumatology, Klinikum Eilbek; 🇩🇪 Hamburg, Germany

The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital; 🇬🇧 Leeds, United Kingdom

Royal Free Hospital, University College London; 🇬🇧 London, United Kingdom