Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis

Abstract

Early intervention in pulmonary arterial hypertension associated with systemic sclerosis (SSc) may improve its prognosis. We aimed to establish an algorithm to detect mean pulmonary artery pressure (mPAP) > 20 mmHg using non-invasive examinations in SSc patients by modifying the DETECT algorithm. This study included SSc patients who underwent right heart catheterization (RHC) in our hospital during 2010–2018. Following variables were assessed for performance to predict mPAP ≥ 25 mmHg or > 20 mmHg; anti-centromere or U1-RNP antibody, plasma BNP level, serum urate level, right axis deviation, forced vital capacity (FVC)/diffusing capacity for carbon monoxide (DLCO) ratio, and tricuspid regurgitation velocity. Of 58 patients enrolled in this study, 24 had mPAP of ≥ 25 mmHg and 9 had mPAP of 21–24 mmHg. Among variables tested, only FVC/DLCO elevated similarly in patients with mPAP of ≥ 25 mmHg (median 2.5) and those with mPAP of 21–24 mmHg (median 2.5) compared to those with mPAP of ≤ 20 mmHg (median 1.5). Given the particularly good correlation between DLCO and mPAP of > 20 mmHg, each variable was weighted according to its odds ratio and the total weighted score was calculated. The total weighted score exhibited a good predictive performance for mPAP of > 20 mmHg with its sensitivity of 87.5% and specificity of 92%. Among conventional risk factors for PAH, decreased DLCO may predict mPAP > 20 mmHg with priority in SSc patients. Weighting DLCO may improve the performance of screening algorithm for early SSc-PAH.

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Acknowledgements

No specific funding was received to carry out the work described in this manuscript.

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All authors were involved in drafting the manuscript or revising it critically for important intellectual content, and approved the final version to be submitted for publication. MK had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study conception and design: HN and MK. Acquisition of data: KN, HN, NA, HO, and IT. Analysis and interpretation of data: KN, HN, MK, YF, KO, SY, and TA.

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Correspondence to Masaru Kato.

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No specific funding was received to carry out the work described in this manuscript. TA reports personal fees from Chugai, during the conduct of the study; grants and personal fees from Astellas, grants and personal fees from Takeda, grants and personal fees from Mitsubishi Tanabe, grants and personal fees from Chugai, grants and personal fees from Pfizer, grants from Daiichi Sankyo, grants from Otsuka, personal fees from Eisai, and personal fees from AbbVie, outside the submitted work. SY reports grants and speaker’s fee from Bristol-Myers Squibb Co., Speaker’s fee from Chugai, Mitsubishi Tanabe, outside the submitted work. MK reports grants from GSK, grants from Actelion, outside the submitted work. The other authors state that they have no conflict of interest. TA reports personal fees from Chugai, during the conduct of the study; grants and personal fees from Astellas, grants and personal fees from Takeda, grants and personal fees from Mitsubishi Tanabe, grants and personal fees from Chugai, grants and personal fees from Pfizer, grants from Daiichi Sankyo, grants from Otsuka, personal fees from Eisai, and personal fees from AbbVie, outside the submitted work. SY reports grants and speaker’s fee from Bristol-Myers Squibb Co., Speaker’s fee from Chugai, Mitsubishi Tanabe, outside the submitted work. MK reports grants from GSK, grants from Actelion, outside the submitted work. The other authors state that they have no conflict of interest.

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Ninagawa, K., Kato, M., Nakamura, H. et al. Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis. Rheumatol Int 39, 1883–1887 (2019). https://doi.org/10.1007/s00296-019-04370-0

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Keywords

  • Pulmonary arterial hypertension
  • Systemic sclerosis
  • Screening
  • FVC/DLCO