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Nailfold videocapillaroscopic changes in patients with pulmonary arterial hypertension associated with connective tissue diseases

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Abstract

Pulmonary arterial hypertension (PAH) represents one of the most devastating complications in connective tissue diseases (CTDs). The aim of this study was to investigate the presence of peripheral microangiopathy in patients with PAH associated with CTDs (CTD-PAH) by exploring nailfold videocapillaroscopic (NVC) changes and identify possible associations of NVC characteristics with markers of disease severity. Α cross-sectional study was performed in 18 CTD-PAH patients [13 PAH due to systemic sclerosis (SSc-PAH) and 5 with other types of CTD-PAH], 14 patients with SSc without PAH (SSc-non-PAH) and 20 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability, capillaroscopic images were reviewed by two independent investigators. When compared to healthy controls, patients with CTD-PAH (77.8% women, mean age 65.9 years) presented reduced capillary density (6.5 ± 1.6 loops/mm vs. 9.7 ± 0.7 loops/mm, p < 0.001) and increased capillary loop width (23.3 ± 10.1 μm vs. 11.2 ± 2.5 μm, p < 0.001). SSc-PAH patients presented lower capillary density in comparison with other CTD-PAH patients and SSc-non-PAH subjects and abnormal and disorganized capillaries compared to controls. Patients with other CTD-PAH had also reduced capillary density and increased loop diameter compared to controls. A significant linear correlation was identified between capillary density and estimated glomerular filtration rate in the total CTD-PAH population (r = 0.63, p = 0.007). In SSc-PAH group, capillary loop diameter was positively correlated to cardiac index (r = 0.61, p = 0.02). Significant NVC microvascular changes were detected in patients with various types of CTD-PAH, suggesting an impaired peripheral microcirculation parallel to pulmonary vasculopathy.

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All data of this study are available upon request.

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Funding

This study received research grant from the Greek Rheumatology Society and Professional Association of Rheumatologists (protocol number 856).

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Authors and Affiliations

  1. Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Street, 54642, Thessaloníki, Greece

    Alexandra Arvanitaki, Eva Triantafyllidou, Eleni Pagkopoulou, Alexandros Garyfallos & Theodoros Dimitroulas

  2. First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, 1 St. Kyriakidi Street, 54636, Thessaloníki, Greece

    Alexandra Arvanitaki, George Giannakoulas & Haralambos Karvounis

  3. Department of Adult Congenital Heart Disease, Royal Brompton and Harefield Foundation Trust, London, Sydney Street, Chelsea, London, SW3 6NP, UK

    Alexandra Arvanitaki

  4. Department of Respiratory Medicine, G. Papanikolaou Hospital, Thessaloníki, Greece

    Afroditi Boutou

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  1. Alexandra Arvanitaki
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  2. George Giannakoulas
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  8. Theodoros Dimitroulas
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Contributions

AA performed the NVC examination, stored and coded the images. AA collected, statistically analyzed and interpreted all data and drafted the manuscript. ET and EP analyzed the NVC images in a blinded manner. GG performed the right heart catheterization and the transthoracic echocardiography. AA, GG and TD conceived the idea, designed the study and critically revised the manuscript. AB, AG and HK critically revised the manuscript for important intellectual content. All the authors read and approved the final manuscript.

Corresponding author

Correspondence to Theodoros Dimitroulas.

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Conflict of interest

AA is the recipient of the International Training and Research Fellowship EMAH Stiftung Karla Voellm, Krefeld, Germany. The rest of the authors declare no conflicts of interest other than the grant received for this research.

Ethics approval

The study received approval from the Aristotle University of Thessaloniki Ethics Committee (protocol number 264) and was performed according to the Declaration of Helsinki.

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Informed consent was obtained from all individual participants included in the study.

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The authors affirm that human research participants provided informed consent for publication of the images in Fig. 2a–f.

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Arvanitaki, A., Giannakoulas, G., Triantafyllidou, E. et al. Nailfold videocapillaroscopic changes in patients with pulmonary arterial hypertension associated with connective tissue diseases. Rheumatol Int 41, 1289–1298 (2021). https://doi.org/10.1007/s00296-021-04839-x

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