Clinical trial NCT01858259: Treatment and Prevention of Progression of Interstitial Lung Disease in Systemic Sclerosis
Official title: Prevention and Treatment of Interstitial Lung Disease in Systemic Sclerosis
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Systemic Sclerosis
Interstitial Lung Diseases
Lead sponsor: Charite University, Berlin, Germany
Sponsor class: OTHER
Collaborators: name: European Union
name: University of Giessen
name: University of Zurich
name: University of Paris 5 - Rene Descartes
name: University of Florence
name: University of Campania Luigi Vanvitelli
name: University of Basel
name: University College, London
name: Charite University, Berlin, Germany
name: University of Pecs
name: University of Leeds
name: Schoen Klinik Hamburg Eilbek
First posted: 2013-05-21
Enrollment: 1372
Sex: ALL
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: The study population are adult and juvenile SSc patients from the EUSTAR cohort (MEDSonline database) and the jSScWG cohort

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.

Eligibility criteria: 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

Primary outcomes:
measure: Proportion of patients with 10% decline in FVC
timeFrame: 1 year
Secondary outcomes:
measure: The time to a 15% decline in DLCO or a drop <55% of predicted lung function
timeFrame: 1 year
measure: The mortality due to lung fibrosis
timeFrame: 1 year
measure: The need for oxygen support
timeFrame: 1 year
Other outcomes:
measure: Identification of confounders
timeFrame: 1 year
measure: Evaluation of the incidence of drug-related adverse events
timeFrame: 1 year
measure: Evaluation of the incidence of withdrawal from treatment due to drug-related adverse events
timeFrame: 1 year
Locations:
city: Paris
country: France
facility: Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016
city: Bad Nauheim
country: Germany
facility: Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology
city: Berlin
country: Germany
facility: Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie
city: Hamburg
country: Germany
facility: Centre for Pediatric Rheumatology, Klinikum Eilbek
city: Pecs
country: Hungary
facility: Pecsi Tudomanyegyetem - University of Pecs
city: Firenze
country: Italy
facility: University of Florence, Denothe Centre, Division of Rheumatology AOUC, Department of Biomedicine
city: Napoli-Italia
country: Italy
facility: Policlinico, Via Pansini
city: Basel
country: Switzerland
facility: Felix-Platter Spital, University of Basel
city: Zurich
country: Switzerland
facility: University of Zurich, Department of Rheumatology
city: Leeds
country: United Kingdom
facility: The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital
city: London
country: United Kingdom
facility: Royal Free Hospital, University College London
Study officials:
affiliation: Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie
name: Gabriela Riemekasten, Prof.
role: PRINCIPAL_INVESTIGATOR
affiliation: Royal Free Hospital, University College London London
name: Christopher Denton, Prof.
role: PRINCIPAL_INVESTIGATOR
affiliation: Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology
name: Ulf Müller-Ladner, Prof.
role: STUDY_CHAIR
References: