Patient selection: scleroderma
Clinical risk factors:
(1) extensive telangiectasia
(2) Raynaud’s phenomenon for > 8 years
(3) positive for anticentromere antibodies
(4) positive for isolated nucleolar antinuclear antibodies (ANA)
(5) limited cutaneous scleroderma
Clinical findings:
(1) dyspnea, especially on exertion
(2) right heart failure
(3) pericardial effusion
(4) murmur of tricuspid regurgitation
where:
• Findings of right heart failure may include edema of the lower extremities, jugular venous distention, hepatomegaly, prominent A wave in the jugular venous pulse, right ventricular heave, etc.
Examination findings:
(1) diffusing capacity of carbon monoxide (DLco) < 60 percent of predicted, in the absence of extensive interstitial lung disease
(2) (forced vital capacity or FVC as percent of predicted) to (DLco as percent of predicted) ratio > 1.6
(3) right ventricular systolic pressure (RVSP) in mm Hg > 40 mm Hg
(4) tricuspid regurgitation jet velocity > 3.0 meters per second
(5) right ventricular dilatation and/or hypokinesis
(6) right atrial dilatation
Laboratory findings:
(1) elevated BNP or NT-proBNP
(2) oxygen desaturation during exercise
