Pulmonologist

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.

Treatment 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.

Some 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.

  1. Vij R, Strek, ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3);814-824.
  2. Fagundes MN, et al. Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med. 2009;103:854-860.
  3. Gomez Carrera L, Bonilla Hernan G. Pulmonary manifestations of collagen diseases. Ach Bronconeumol. 2013;49(6):249-260.
  4. Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3);814-824.

Provided by Prof. Heussel and PD Kreuter (Thoraxklinik Heidelberg, Germany)
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