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İstanbul Üniversitesi / Cerrahpaşa Tıp Fakültesi / Göğüs Hastalıkları Anabilim Dalı

Akciğer tutulumu olan sistemik skleroz olgularının özellikleri

The features of systemic sclerosis patients with pulmonary involvement

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SUMMARY 56 female and 5 male scleroderma patients with pulmonary involvement were recruited in our study. Their mean age was SO ± 12 years.They had an average disease duration of 10 ± 8 years and exertional dyspnea for 3 ± 3 years. 44% of patients had stage 2 dyspnea score. On physical examination, 82% of the patients had late inspiratory crackles. Pulmonary function test results were as follows: FVC: 2107 ± 696 ( 72% ± 19), FEV1: 1812 ± 643 (76% ± 18), FEV1 / FVC: 83% ± 8, MMFR 2,3 ± 1,1(76% ± 29), DLCO: 1 1,3 ± 5(49% ± 17). 62% of the patients showed restrictive type respiratory impairment. All patients had impairment in diffusion capacity. Radiological evaluation showed that 1 1% of the patients had normal chest x-ray. 87 % had reticulonodular and 3 % had honeycomb lesions When high resolution computerized tomography (HRCT) were applied to the patients, the distribution of pathological appearance were as follows: ground glass= 10%, reticular images= 14%, mixed lesions= 76%. Bronchoalveolar lavage showed neutrophilic, lymphocytic and mixed type of alveolitis in 58%, 3% and 39% of patients respectively. Pulmonary artery pressure (PAP) measurements were performed in 26 patients.Mean systolic pulmonary artery pressure was 35 ± 15 mmHg and 73% of patients had pulmonary hypertension( PAP > 25 mmHg ) *When the results were evaluated, dyspnea score showed negative correlation with diffusion capacity (p: 0,0031) and FVC (p: 0,01). A similar correlation was also true between duration of dyspnea and FVC (p: 0,01), DLCO ( p: 0,006). Patients with late inspiratory crackles had significant correlation with FVC ( p: 0,045), DLCO (p: 0,0013).As duration of dyspnea got longer, having crackles was more likely to occur. We could not detect any correlation between bronchoalveolar lavage (BAL) and clinical parameters. Pulmonary artery pressure showed negative correlation with forced vital capacity. As a conclusion we have seen that BAL has little value in evaluating the stage of disease and interpreting the prognosis. For disease follow - up, spirometric and diffusion capacity measurements offer more reliable and valuable results. HRCT happens to be more advantageous and sensitive to chest x-ray, showing interstitial lesions at an earlier stage. Moreover Raynaud's phenomenon was the initial symptom for all our patients. We thought that this symptom should be alarming to start evaluating the possible involvement of the lungs. Further studies can be concentrated on the early detection of the disease which may add to the understanding of the disease and response to therapy. 4*