Answer and References

Answer: A

A.      A ratio of FVC/DLCO percent predicted >1.6 suggests PH. 

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DLCO <65% predicted in the absence of significant lung volume abnormalities or ≥20% decrease in DLCO over the course of a year suggests PH, particularly in a patient with longstanding limited cutaneous SSc. The FVC/DLCO ratio is another way to assess whether the degree of impairment of gas transfer is greater than what would be expected on the basis of ILD.

B.      FVC and/or total lung capacity <80% predicted with reduced DLCO is indicative of a restrictive pattern, consistent with ILD. ILD and PH may occur simultaneously, but this pattern on pulmonary function testing alone is not highly suggestive of PH.

C.      Plasma levels of BNP and/or NT-proBNP are useful screening tools for PH.  In one study, elevated NT-proBNP levels in patients with SSc patients had a sensitivity and specificity of 90% for the presence of pulmonary arterial hypertension. 


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