Screening for pulmonary arterial hypertension with echocardiography is warranted in patients with scleroderma and mixed connective tissue disease
Design
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Multicenter, prospective, retrospective study to determine prevalence of undiagnosed
PAH in 50 community-based rheumatology practices
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Patients
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909 patients with either scleroderma (SSc) or mixed connective tissue disease (MCTD)
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Key findings
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13.3% of SSc and MCTD patients were found to have undiagnosed PAH using Doppler echocardiography as a screening tool.
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More than 20% of these patients had evidence of advanced disease.
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Study limitations
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Two centers enrolled more patients than others.
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Participating rheumatologists were aware of the goal to discover possible PAH.
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No central laboratory was used to evaluate Doppler echocardiography results.
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Right heart catheterization was used in only a small minority of patients.
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Conclusions
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Findings suggest that echocardiography screening is justified in patients with SSc
and MCTD, irrespective of symptoms.
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Tracleer is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) in
patients with WHO Class II-IV symptoms, to improve exercise ability and decrease the rate of
clinical worsening. Patients with WHO Class II symptoms showed reduction in the rate of clinical
deterioration and a trend for improvement in walk distance. Physicians should consider whether
these potential benefits are sufficient to offset liver injury in WHO Class II patients, which may
preclude future use as their disease progresses.
Important safety information
Because of the associated risks, Tracleer may be prescribed only through the Tracleer Access Program.
Potential for serious liver injury (including, after prolonged treatment, rare cases of liver failure
and unexplained hepatic cirrhosis in a setting of close monitoring)—Liver monitoring of all patients
is essential prior to initiation of treatment and monthly thereafter.
High potential for major birth defects—Pregnancy must be excluded and prevented through the use of
reliable forms of birth control; monthly pregnancy tests should be obtained.
Contraindicated for use with cyclosporine A and glyburide.
Please see full prescribing information including BOXED WARNING.
- Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. The prevalence of
undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease
at the secondary healthcare level of community-based rheumatologists (the UNCOVER
study). Arthritis Rheum. 2005;52:2125-2132.
