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Dr. Krasuski:
Connective Tissue Diseases. Identifying Patients with Pulmonary Hypertension. Patients with systemic sclerosis can have different reasons for their pulmonary hypertension. As you can see, there's tremendous crossover here. They can have Group 1, or PAH. There can be patients with significant interstitial lung disease, and those folks fall predominantly into Group 3. They can also develop Group 2 disease because it's well known that myocardial problems also can occur. So there's a tremendous overlap that eventually leads to right heart failure.
Here is an example of the different types of pulmonary hypertension in systemic sclerosis. On the left you can see a patient with interstitial lung disease. In this case, it's much more diffuse than limited. Their very abnormal lung architecture is present. There's reduced vital capacity and these patients are chronically hypoxic. There's also normal to moderate PA pressure elevation. So these are patients who predominantly have an ILD component with some pulmonary hypertension. On the right, on the other hand, is somebody with pulmonary arterial disease. In this case limited considerably is greater than the diffuse type of disease. There is normal or minimally abnormal lung architecture. There's mild basilar fibrosis often. There's very low DLco and a normal vital capacity. And there's evidence of right ventricular dysfunction. The heart typically becomes significantly greater in size and there's elevated PA pressures. In this case, you have rather significant pulmonary hypertension.
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