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Overview of Pulmonary Hypertension in Systemic Scleroderma Presented by Amanda Thorpe
Pulmonary Hypertension is not the same as "regular" high blood pressure or hypertension, which can be easily measured with a blood pressure test.
Pulmonary hypertension is a high pressure in the arteries between the heart and lungs or pulmonary arteries. It is very difficult to detect elevation in pressure in these arteries. Pulmonary Hypertension is a type of vascular disease that can be found in scleroderma patients. (Also see: Scleroderma Lung Involvement, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)
Causes of Pulmonary Hypertension
Causes of Pulmonary Hypertension. There are dozens of causes of pulmonary hypertension, ranging from genetics, medications or street drugs, connective tissue diseases (such as scleroderma), heart and lung diseases, and blood disorders. ISN.
When PH occurs by itself, it is called
Primary Pulmonary Hypertension.
Primary pulmonary hypertension occurs by itself and not secondary to another illness, such as scleroderma. Nailfold capillary examination can easily tell whether pulmonary hypertension is primary or secondary. (Also see: Nailfold Capillaroscopy)
Secondary Pulmonary Hypertension
Treatments and their effectiveness can vary
depending on whether PH is primary or secondary.
Secondary Pulmonary Hypertension. When PH occurs along with other lung, heart, or systemic connective tissue disease (such as scleroderma), it is called Secondary Pulmonary Hypertension. The distinction is important because treatments, and their effectiveness, can vary depending upon whether the pulmonary hypertension is primary or secondary. ISN.
Isolated Pulmonary Hypertension in Systemic Sclerosis)
Isolated Pulmonary Hypertension. In systemic scleroderma, pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) often occurs secondary to severe pulmonary fibrosis, kidney failure, or cardiomyopathy with diastolic dysfunction. When it occurs in scleroderma by itself, and not secondary to pulmonary fibrosis or kidney failure, it is referred to as isolated pulmonary hypertension. ISN.
Exercise-induced pulmonary hypertension common in systemic sclerosis. It is extremely important that these high risk patients (limited scleroderma with long-standing disease, patients with anti-centromere antibody, or a nucleolar pattern on ANA testing) have yearly pulmonary function tests and echocardiograms. MedicExchange. 08/06/08.
Pulmonary Hypertension in Correlation with Other Diseases
Renal disease in systemic sclerosis with normal serum creatinine. A positive correlation was reported between the presence of renal involvement and pulmonary vascular involvement. Patients with systemic sclerosis should be screened for renal involvement irrespective of disease severity or duration. Reem H. Mohamed. (SpringerLink) Clinical Rheumatology. 2/22/10. (Also see: Renal Involvement)
Diagnosis of Pulmonary Hypertension
Diagnosis of Pulmonary Hypertension. Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. ISN.
Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN.
Treatments include oxygen therapy, Tracleer (bosentan), Digitalis, diuretics, Flolan (in clinical trials), and, in a few cases, heart-lung or double-lung transplants.
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