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

Prevention and Treatment of Interstitial Lung Disease in Systemic Sclerosis

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

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.

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