Background: Systemic sclerosis (SSc) is characterized by inflammation, vasculopathy and progressive fibrosis. Pulmonary Hypertension (PH) is one of the leading causes of death in SSc (1). Currently, most patients with SSc are screened for the presence of PH, and focus lies on early detection and early treatment. Recent literature describes potential for both nailfoldcapillaroscopy (NCM) and several biomarkers to serve as non-invasive tools to identify SSc patients at risk for developing PH (2). Ideally this could attribute to further improvement of risk stratification in SSc and PH screenings algorithms.

Objectives: To explore NCM characteristics and plasma levels of selected candidate biomarkers in a cross-sectional cohort of SSc patients with and without different forms of PH.

Methods: From 02-2018 until 02-2019 we included 40 consecutive SSc patients with associated PH (30 (75%) were female, 32 (80%) LcSSc, median age 72 years (IQR 69-77), median SSc duration 10.7 years (IQR 4.3-17.8), median PH duration 3.9 years (IQR 1.5-7.06)) and 40 without PH (28 (70%) were female, 26 (65%) had a LcSSc, median age 59 years (IQR 51-71), median SSc duration 6 years (IQR 3.1-8.6). In each group NCM characteristics (both quantitative and qualitative) and plasma levels of IL4, IL6, IL8, IL13, PDGFAA, PDGFAB-BB, 6Ckine, sTRAIL, MMP1, MMP7, sICAM1, sVCAM, CCL19/MIP3b, Endostatin, sVEGFR1, sVEGFR2, sVEGFR3, CXCL4, Endothelin1, FGF1, FGF2, VEGF-A, VEGF-C, and VEGF-D using Luminex kits, and vascular auto-antibodies AT1R and ETAR using ELISA (Celltrend GmbH, Luckenwalde, Germany) were determined. NCM characteristics were compared using t-tests, biomarker levels were compared by using Mann-Whitney U tests.

Results: We observed no differences in mean capillary density, number of abnormally shaped capillaries, number of fingers with density <3 capillaries/mm and in overall NCM pattern between patients with and without PH. Plasma levels showing significant differences between the two groups are presented in table 1 .

Conclusion: We found significant differences in several of the selected biomarkers in SSc patients with and without PH, but not in NCM characteristics between the groups. However, we did observe a tendency toward more morphologic abnormalities and an overall late pattern in the SSc-PH group. Future longitudinal research should explore the added value of these NCM parameters and biomarkers in personalized risk stratification for the development of PH.

REFERENCES:

[1]Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis. 2017;76(11):1897-905.

[2]Guillén-Del-Castillo A, Simeón-Aznar CP, Callejas-Moraga EL, Tolosa-Vilella C, Alonso-Vila S, Fonollosa-Pla V, et al. Quantitative videocapillaroscopy correlates with functional respiratory parameters: a clue for vasculopathy as a pathogenic mechanism for lung injury in systemic sclerosis. Arthritis Res Ther. 2018;20(1):281.

Disclosure of Interests: Jacqueline Lemmers: None declared, C.H.M. van den Ende: None declared, R. Smeets: None declared, Brigit Kersten: None declared, Arjan van Caam: None declared, Sander van Leuven: None declared, Jolanda van Haren-Willems: None declared, Arie van Dijk Speakers bureau: Actelion(Janssen), Consultant of: Actelion(Janssen), Grant/research support from: Unrestricted educational Grant for PhD student from Actelion (Janssen), Madelon Vonk Speakers bureau: Actelion(Janssen), Boehringer Ingelheim, Roche, Consultant of: Advisory Board from Actelion(Janssen) and Boehringer Ingelheim, Grant/research support from: Unrestricted Educational Grant and research support from Actelion(Janssen), research support from Ferrer