Fischer et al developed an algorithm for identifying 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
Parameters:
(1) clinical risk factors
(2) diffusing capacity of carbon monoxide (DLco) as percent of predicted, in the absence of extensive interstitial lung disease
(3) (forced vital capacity or FVC as percent of predicted) to (DLco as percent of predicted) ratio
(4) right ventricular systolic pressure (RVSP) in mm Hg
Clinical risk factors:
(1) dyspnea
(2) Raynaud’s phenomenon for > 8 years
(3) positive for anticentromere antibodies
(4) positive for isolated nucleolar antinuclear antibodies (ANA)
|
Number of Risk Factors |
DLco |
FVC to DLco ratio |
RVSP in mm Hg |
Risk |
|
none |
>= 70% |
<= 1.6 |
< 35 |
low |
|
1 or more |
>= 70% |
<= 1.6 |
< 35 |
mild |
|
1 or more |
< 70% |
> 1.6 |
> 35 |
moderate |
|
1 or more |
< 60% |
> 1.6 |
> 40 |
high |
A dyspneic patient with one or both of the following should undergo right heart catheterization even if the RVSP is not elevated:
(1) right ventricular hypokinesis or dilatation on echocardiography
(2) increased BNP or NT-proBNP
|
Risk |
Action Plan |
|
low |
Repeat pulmonary function testing annually; repeat echocardiogram in 2-3 years. |
|
mild |
Repeat pulmonary function testing annually; repeat echocardiogram every year. |
|
moderate |
Repeat echocardiogram in 3-6 months or proceed to right heart catheterization. |
|
high |
Proceed to right heart catheterization. |
Specialty: Pulmonology, Cardiology
ICD-10: ,
