CONNECTIVE TISSUE DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE: CASE REPORTS

Abstract

Interstitial lung diseases (ILD) or diffuse parenchymal lung diseases (DPLD) account for approximtely 15% of all pulmonary pathology in clinical practice. These include over 200 diseases, many of which are rare and of unknown etiology. Systemic connective tissue diseases (SCTDs) are a heterogeneous group of rheumatic diseases. They are characterized by various organs systems involvement with autoimmune phenomena and positive autoantibodies. Pulmonary manifestations represent one of the multiple organ involvements. ILD in SCTDs is characterized by inflammation and subsequent development of interstitial pulmonary fibrosis, progressing to impaired gas exchange.

We present here a patient with long-term systemic sclerosis, a patient with antisynthetase syndrome and a patient with systemic lupus erythematosus with rheumatoid arthritis and concomitant ILD. Clinical manifestations are described in details, high-resolution CT scans of the lungs are presented. Two-years methylprednisolone and cyclophosphamide pulse therapies were administered in systemic sclerosis patient, and systemic lupus and antisynthetase syndrome patients underwent methylprednisolone pulse therapy for two years. This treatment was followed by chronic administration of corticosteroids, Imuran and chloroquine. Patients with SCTDs and ILD require a multidisciplinary approach, including clinical, radiographic and histological testing.