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• W2140034345 abstract "Background: The aim of the present study was to evaluate the role of non-invasive methods in the early detection of pulmonary and cardiac involvement in Systemic sclerosis (SSc) and to identify clinical and/or instrumental patterns of prognostic value. Patients and Methods: Twenty female patients affected by SSc (8 with diffuse cutaneous SSc and 12 with limited cutaneous SSc) were enrolled in our study. Cardiac and pulmonary involvement (respiratory function tests and carbon monoxide lung diffusion (DLCO ), chest radiography, high resolution computed tomography (HRCT) and lung perfusion magnetic resonance) were evaluated. Results: All 18 patients studied with respiratory function tests showed a significant reduction of DLCO. HRCT was considerably more sensitive than traditional chest radiography (59% versus 28%; p<0.05). Lung perfusion MRI revealed normal findings in 15 patients. Abnormal lung perfusion MRI results were found only in 3 patients. Angina pectoris with electrocardiographic and scintigraphic ischemic changes, severe regional wall motion abnormalities and complex arrhythmias seemed to be associated with poor prognosis. Conclusion: Taken together these results indicate that a pulmonary involvement occurs both in limited and in diffuse cutaneous SSc patients and develops, in 83 % of the cases, without any regional lung perfusion abnormality. Furthermore, cardiac involvement is detected in 65 % of the cases as a consequence of a range of noxious events including myocardial ischemia, fibrosis and pressure overload which may result in ventricular dysfunction and arrhythmias. Lung perfusion MRI should be considered as a complementary diagnostic method for the functional evaluation of these symptoms in systemic sclerosis. Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology characterized by vascular pathology, especially of the microvasculature, and tissue fibrosclerosis with involvement of the skin, gastrointestinal tract and organs such as the lungs, heart and kidneys. In a recent study based on the Pittsburgh Data Bank (20), renal involvement was observed in 19%, cardiac in 15% and pulmonary in 16 % of 953 patients. The improved survival obtained in the renal crisis by effective therapy with ACE- inhibitor drugs has made pulmonary fibrosis the most frequent cause of death in SSc, nowadays (7, 19-20). During the first five-year period from the onset of the disease, 44% of deaths had a cardiac or renal origin compared to 18% in the successive five-year period, with an overall 40% cardiac mortality. Furthermore, a higher mortality rate due to pulmonary fibrosis was present in the second five-year period (32%) when compared to the first five-year period (9%), corresponding to an overall 41% of total deaths (20). These data demonstrate that, in the heart and in the kidneys, the progression of the functional alterations can lead more rapidly to severe disease and death while the pulmonary damage, although occurring early, proceeds asymptomatically toward a large loss of pulmonary volume. These observations point out the significance of the early detection of organ damage in modifying the course of the disease through an appropriate treatment. Cardiac involvement is present in more than 80% of SSc patients and consists of various pathological changes including hypertrophy, inflammation and myocardial fibrosis, occasionally associated with segmental necrosis of the myocytes and contraction band necrosis due to severe ischemia and reperfusion injury, despite the absence of obstructive coronary lesions (8, 11, 12, 20, 22, 27) ." @default.
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• W2140034345 date "2004-03-01" @default.
• W2140034345 modified "2023-09-23" @default.
• W2140034345 title "Non-invasive diagnostic and functional evaluation of cardiac and pulmonary involvement in systemic sclerosis." @default.
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