Fischer et al listed clinical and laboratory findngs in a patient with scleroderma-associated pulmonary arterial hypertension (PAH). The authors are from the University of Colorado in Denver and Georgetown University in Washington, D.C.


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



• 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


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