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| |  ACR: Pulmonary Hypertension Prevalent in Patients With Connective Tissue Disease By Bruce Sylvester SAN ANTONIO, TEXAS -- October 20, 2004 -- Potentially fatal pulmonary hypertension (PAH) often goes unrecognized and untreated in patients with scleroderma and mixed connective tissue disease (CTD), researchers reported here today at a press conference at the 68th Annual Scientific Meeting of the American College of Rheumatology. "It's a killer that we often miss," said investigator Frederick M. Wigley, MD, professor of medicine, rheumatology division, Johns Hopkins University, Baltimore, Maryland. "Pulmonary hypertension is easy to overlook in simple examinations, and can progress to [an] irreversible state. It is more prevalent in patients with scleroderma and CTD than we knew." Dr. Wigley and colleagues completed a chart review of 791 patients with scleroderma or mixed CTD from 50 medical practices. The 669 patients who showed no previous history of pulmonary hypertension were enrolled in a prospective study in which clinicians performed Doppler echocardiogram (ECHO) to assess estimated right-ventricular systolic pressure (ERVSP). The presence of pulmonary hypertension was diagnosed when right-ventricular systolic pressure was greater than 40 mmHg. Subjects also completed exercise-tolerance questionnaires, with ratings in 3 domains from 0 to 4 (0 = severe and 4 = no impairment). In the final analysis, 13.3% of the patients had an ERVSP > 40 mmHg on ECHO. ECHO also indicated 22.5% of subjects with an ERVSP of of 50 mmHg or greater, 22.5% with an increased RV dimension, and 28.1% with RA enlargement and an LV ejection fraction of 61.8. The researchers found that patients with ERVSP > 40mmHg (27%) reported decreased tolerance to exercise compared to subjects with < 40mmHg (9.5%). The authors concluded that the ECHO evaluation of patients with connective tissue disease is important for the detection of patients who may require further evaluation and care for pulmonary hypertension. These conclusions are based on the results of the Undiagnosed Pulmonary Hypertension in Patients with Connective Tissue Disease attending Community Based Rheumatology Clinics (UNCOVER) Study. "Doctors need to think in terms of regular screenings for at-risk patients," added Dr. Wigley, "since early detection with ECHO technology makes it possible to intervene with new available vasoactive medications that have the potential to prevent disease progression and possible fatality."
[Presentation title: The Point Prevalence of Undiagnosed Pulmonary Hypertension (PAH) in Patients with Connective Tissue Disease (CTD) Attending Community Based Rheumatology Clinics (UNCOVER Study). Abstract 1057]
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