Patients with systemic sclerosis are at high risk for developing interstitial lung disease, which is one of the main reasons for death in these patients. Patients with systemic sclerosis associated interstitial lung disease have a median survival of five to eight years.
A cofactor might be oesophageal involvement, as there is some evidence that gastroesophageal reflux might cause or at least aggravate interstitial lung disease.
of systemic sclerosis associated interstitial lung disease or other
connective tissue disease associated lung diseases mainly comprises of
immunosuppressive therapy. However, the benefits and efficacy of this
therapy still remain controversial.
data suggest that therapy with cyclophosphamide, mycophenolate mofetil
or rituximab (besides others) might be beneficial. Further controlled
prospective trials are still going on comparing efficacy of
mycophenolate or rituximab in these patients.
- Vij R, Strek, ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3);814-824.
- Fagundes MN, et al. Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med. 2009;103:854-860.
- Gomez Carrera L, Bonilla Hernan G. Pulmonary manifestations of collagen diseases. Ach Bronconeumol. 2013;49(6):249-260.
- Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3);814-824.