Session Type: Abstract Submissions (ACR)
Background/Purpose: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in systemic sclerosis (SSc). Pulmonary arterial hypertension (PAH) is reportedly the most frequent cause of PH in SSc, affecting 8-12% of patients. We observed that a significant proportion of our SSc patients with PH as diagnosed by right heart catheterization (RHC) had PH due to causes other than PAH. The frequency of other causes of PH in SSc has not been accurately determined to date. The aim of the present study was therefore to determine the frequency of different causes of PH in our SSc cohort and to identify associated clinical, serological and radiologic variables.
Methods: A retrospective analysis of 432 SSc patients was done. All patients routinely underwent screening for PH. Clinical, serological and radiographic data from patients with PH confirmed by RHC (n=26) were analyzed. Living SSc PH patients (n=21) and 19 control SSc patients without clinical PH were prospectively re-evaluated with serial measurements of NT-proBNP and the hemodynamic biomarkers MR-proANP and MR-proADM.
Results: The most frequent cause of PH was left heart disease (LHD) (15/26, 58%). PAH was seen in 9/26 patients (34%). Other causes of PH were veno-occlusive disease and multifactorial PH. No association was found between the type of PH and the autoantibody profile. LHD-related PH was associated with significantly lower NT-proBNP levels than PAH at the time of established PH (137 ± 137 pg/ml vs 484 ± 248 pg/ml, p=0.024), and all SSc patients without PH had normal levels of NT-proBNP. MR-proANP and MR-proADM levels were significantly higher in SSc patients with PH than those without PH (105 [81-151] pmol/L vs 78 [32-91] pmol/L, respectively, p = 0.004, and 0.7 [0.42-0.87] nmol/L vs 0.5 [0.4-0.6] nmol/L, p = 0.018,). Similarly, MR-proANP and MR-proADM levels were significantly higher in SSc patients with LHD-PH as compared to those without PH (105 [87-137] pmol/L vs 78 [32-91] pmol/L, respectively, p = 0.014, and 0.75 [0.47-0.92] nmol/l vs 0.5 [0.4-0.6] nmol/L, p= 0.012). However, there was no significant difference between the LHD-PH and the PAH groups.
Conclusion: SSc-associated PH is heterogeneous. RHC is essential to determine the underlying cause. The most frequent cause of PH is LHD, not PAH. Levels of MR-proANP and MR-proADM, but not NT-proBNP, were increased in LHD-PH, and may be more reliable markers than NT-proBNP in this subgroup of patients. This study is the first to identify such a high frequency of LHD-PH correlating with normal NT-proBNP levels but increased MR-proANP and MR-proADM in SSc.
J. R. Goulet,
J. L. Senécal,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/left-heart-disease-is-a-frequent-cause-of-pulmonary-hypertension-in-systemic-sclerosis-is-associated-with-increased-levels-of-mr-proanp-and-mr-proadm-but-is-unrelated-to-elevated-nt-probnp-levels-a/