Akciğer tutulumlu sklerodermalı olgularda fonksiyonel parametrelerin değerlendirilmesi
Özet
Pulmonary involvement of scleroderma really worsens the prognosis of the disease. With the recent development of ACE inhibitors, the mortalities due to renal crisis were decreased. This lead to the accusation of pulmonary involvement which is the leading cause for mortality in scleroderma. Since early diagnosis and treatment are priceless, the patients who were diagnosed as scleroderma must be assessed for pulmonary involvement. In this study we evaluated certain tests for scleroderma and we investigated the correlation between those tests.33 scleroderma patients with pulmonary involvement from immunology and rheumatology departments or from pulmonary diseases departments of Ankara University School of Medicine, who were admitted between 2005 and 2007, were included to the study. Datas of the patients were examined prospectively. When the patients were evaluated radiologically, pathological findings were observed in thorax HRCT, even when their chest X-rays were normal. The first disturbed PFT parameter was found to be %DLCO.Statistically significant relationship was observed between sPAP, dyspnea scores, and functional classification. The negative correlation between %DLCO and MRC dyspnea score and NYHA functional clasification shows that the scores can be used in early diagnosis.6MWT found to be a valid test for both types of scleroderma (interstitial lung disease type and pulmonary hypertension type). The inverse relation between 6MWT and dyspnea scores and functional classification lead to the belief that 6MWT is correlated with the severity of disease and is valid for evaluating the exercise capacity in this patient group.Although SGRQ was not originally designed for scleroderma patients, it was thought to be a useful tool for reflecting the performance status of the patients and determining the degree of disease.To conclude, scleroderma patients must be assessed for pulmonary involvement, even if they are asymptomatic, and they must be followed up. Pulmonary function tests, echocardiography, thorax HRCT, 6MWT are valuable and irreplaceable tests for diagnosis and follow up. Also, the patients must be classified functionally according to WHO and MRC and BORG dyspnea scored must be calculated. They are helpful for an early diagnosis. SGRQ quality of life questionnaire is a recent noninvasive parameter in diagnosis and follow up which is correlated with the other tests.Key Words: Scleroderma, PHT, DLCO, 6MWT, SGRQ