Interstitial Lung Diseases, Systemic Sclerosis
Conditions
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.
Interventions
None listed
Sponsors
European Union
University of Giessen
University of Zurich
University of Paris 5 - Rene Descartes
University of Florence
University of Campania Luigi Vanvitelli
University of Basel
University College, London
Charite University, Berlin, Germany
University of Pecs
University of Leeds
Schoen Klinik Hamburg Eilbek
Gabriela Riemekasten
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame |
|---|---|
| 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 |
| The need for oxygen support | 1 year |
Other
| Measure | Time frame | Description |
|---|---|---|
| Evaluation of the incidence of drug-related adverse events | 1 year | — |
| Evaluation of the incidence of withdrawal from treatment due to drug-related adverse events | 1 year | — |
| Identification of confounders | 1 year | Parameters with impact on ILD progression independent of therapies, such as SSc subgroups, presence of antibodies, presence of vasculopathy, time to therapy initiation, degree of lung fibrosis defined by FVC values, age at disease onset and at treatment initiation, gender, co-morbidities and other confounders. |
Countries
France, Germany, Hungary, Italy, Switzerland, United Kingdom
Contacts
Primary ContactGabriela Riemekasten, Prof.
Backup ContactChristopher Denton, Prof.
Outcome results
None listed