Newswise — Pulmonary hypertension, which is high blood pressure in the lungs, is often not diagnosed in patients with scleroderma and mixed connective tissue disease despite its being a major disease complication and leading cause of death in this patient population, according to research presented this week at the American College of Rheumatology Annual Scientific Meeting in San Antonio, Texas.
Scleroderma and mixed connective tissue disease are two types of rheumatic autoimmune diseases that cause inflammation, damage and scarring in the skin and internal organs, including the lung and its blood vessels. Pulmonary hypertension, a common and devastating complication of these diseases, results in the death of about half of these patients within two to three years after diagnosis. However, the onset of pulmonary hypertension may be without signs and symptoms, like tiredness or shortness of breath. Therefore, diagnosis is often difficult and very little information exists on the prevalence of pulmonary hypertension in patients followed in community practice.
To assess the prevalence of pulmonary hypertension in people with scleroderma and mixed connective tissue disease, researchers studied patients, drawn from 50 medical practices in North America, who had no previous pulmonary hypertension diagnosis. Patients underwent a Doppler echocardiogram (ECHO) of the heart and exercise tolerance questioning. Pulmonary hypertension was considered present if the ECHO determination of estimated right ventricular systolic pressure was greater than 40mmHg. (Systolic pressure, which is the first number on a blood pressure reading, represents the maximum pressure exerted when the heart contracts. A high systolic pressure indicates strain on the blood vessels when the heart is attempting to pump blood into the bloodstream.)
Of the 669 patients who had no previous diagnosis of pulmonary hypertension, 89 (13.3 percent) registered a greater than 40mmHg reading, showing the presence of pulmonary hypertension. This significant number suggests that an echocardiogram evaluation of patients with scleroderma or mixed connective tissue disease should be conducted regularly to detect any need for further evaluation or intervention therapy for pulmonary hypertension. Results also showed that only a minority of these patients ever had a workup to look for pulmonary hypertension.
"Pulmonary hypertension is a silent killer of these patients, difficult to detect by simple bedside examination until the late irreversible stage is present," said Fredrick M. Wigley, MD, Rheumatology Division, Johns Hopkins University, Baltimore, Maryland, and an investigator in the study. "Patients should be screened regularly because early detection with ECHO technology provides the opportunity to treat with new available vasoactive medications that potentially can prevent progression to severe life-threatening disease."
The American College of Rheumatology is the professional organization for rheumatologists and health professionals who share a dedication to healing, preventing disability and curing arthritis and related rheumatic and musculoskeletal diseases. For more information on the ACR's annual meeting, see http://www.rheumatology.org/annual.
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