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Issues remain in treatment, diagnosis of PH in patients with inflammatory rheumatic diseases
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Unresolved issues were present in the treatment and diagnosis of early pulmonary hypertension in patients with inflammatory rheumatic diseases, who are at higher risk for pulmonary arterial hypertension, according to researchers at the Hacettepe University Faculty of Medicine in Ankara, Turkey.
Of the 50 patients with rheumatic diseases studied, 34 had systemic sclerosis (SSc); seven had systemic lupus erythematosus (SLE); four had rheumatoid arthritis (RA); two had sarcoidosis; and one each had Sjögren's syndrome (SS), undifferentiated collagen tissue disease or Takayasu's arteritis. Mean age was 52.1 years, and 42 of the patients were women.
Doppler echocardiography revealed mean systolic pulmonary arterial pressure (sPAP) of 59.9 mm Hg. Four patients had sPAP below 40 mm Hg. Among all patients, left ventricular ejection was less than 50%, and the right ventricular diameter was increased by more than 2.5 cm in 31 patients. Pericardial effusion on transthoracic echocardiograph was present in 19 patients.
Right heart catheterization was scheduled for 47 patients, two of whom died before the procedure (one with RA and one with SSc) and one of whom refused the procedure. Adenosine or inhaled iloprost were used to assess acute vasodilator testing during the procedure.
Pulmonary arterial hypertension (PAH) was shown to cause pulmonary hypertension (PH) in 40.4% of patients, whereas chronic thromboembolic was the cause of PH in five patients.
At the time of diagnosis, the New York Heart Association Functional Classification was III or IV in 79% of patients with PAH, and the researchers suggested that better methods are needed to identify PH in patients at an earlier stage. - by Shirley Pulawski
Disclosure: The researchers report no relevant financial disclosures.
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