Ab0607 analysis of nailfold capillaroscopy findings and clinical features of patients with systemic lupus erythematosus and pulmonary arterial hypertension

Background Systemic Lupus Erythematosus (SLE) is an autoimmune disease affecting different organs and causing significant morbidity and mortality. Pulmonary arterial hypertension (PAH) is a rare manifestation of SLE. The prevalence of PAH in patients with SLE varies between 0.5 to 5% [1] . No screening algorithms have been developed in patients with SLE. In patients with SSc, PAH is characterized by changes in the pulmonary vasculature and endothelial dysfunction. These microvascular changes are also present in periphery, observed with nailfold video-capillaroscopy (NVC) [2] . NVC findings may help to identify patients at a significant high risk of future development of PAH [3] . Objectives The aim of our work is to analyze the clinical and demographic features and nailfold capillary changes of patients with SLE-related PAH compared to a group of SLE patients without PAH. Methods We identified and selected 20 patients with SLE and type I PAH and collected demographic, clinical and laboratory features from 8 rheumatology centers across Europe. We could perform NVC on 9 patients. We selected as controls 68 patients with SLE who underwent cardiopulmonary screening to exclude PAH: we collected demographic, clinical and laboratory features and performed NVC. The presence of SD pattern was assessed as previously described [4] . Patients satisfied the 2019 EULAR/ACR SLE classification criteria. We excluded patients with a diagnosis of mixed tissue disease and overlap syndrome. Results Demographic and clinical features of patients with SLE-PAH and SLE controls are shown in Table 1. All patients with SLE-PAH were female, age and disease duration were not different between the 2 groups. LAC+ and anti-RNP+ was more prevalent in patients with SLE-PAH. No differences were observed for anti-Sm, anti-Ro, anti-La and anti-phospholipid antibodies. Of clinical features, skin and CNS involvement were more prevalent in patients with SLE-PAH than in SLE controls. Raynaud’s phenomenon was more prevalent in patients with SLE-PAH than in SLE controls. In patients with SLE-PAH we observed a significantly higher prevalence of scleroderma pattern at NVC than in controls: patients with SLE-PAH showed a lower number of capillary density and a higher frequency of megacapillaries. In multivariate analysis, Raynaud phenomenon and anti-RNP are predictors of PAH in patients with SLE. The McFadden’s R-squared for the model is 0.30. Conclusion Our data show that LAC+, RNP+, Raynaud’s, Skin and CNS involvement and a SD pattern at NVC is more prevalent in patients with SLE PAH than in patients with SLE without PAH. Our results point to a generalized microvascular involvement and a hypercoagulation state in patients with SLE PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE with a high risk of developing PAH. References [1]Bazan, Resp medicine, 2018 [2]Corrado, Microvascular research, 2017 [3]Pauling, Rheumatology, 2017 [4]Smith, Autoimmunity Reviews, 2019 Table 1. Demographic and clinical characteristics of study population SLE no PAH n=68 SLE PAH n=20 p-value Age, years (median [IQR]) 43.80 [33.48, 52.83] 42.30 [34.32, 49.71] 0.46 Female sex, n (%) 49 (72.1) 20 (100.0) 0.01 Disease duration, years (median [IQR]) 13.35 [6.35, 19.53] 17.40 [7.33, 20.77] 0.34 Serology, n (%): anti-Sm 13 (19.1) 6 (30.0) 0.46 anti-RNP 12 (17.6) 9 (45.0) 0.02 anti-Ro 32 (47.1) 10 (50.0) 1 anti-La 5 (7.4) 3 (15.0) 0.54 LAC 13 (19.1) 9 (45.0) 0.03 anti-β2GPI IgG/IgM 11 (16.2) 2 (10.5) 1 anti-CL IgG/IgM 14 (20.6) 5 (25.0) 0.75 Clinical, n (%): Mucocutaneous 25 (36.8) 13 (65.0) 0.04 Serositis 11 (16.2) 7 (35.0) 0.12 Heamatological 38 (55.9) 15 (75.0) 0.20 Musculoskeletal 33 (48.5) 9 (47.4) 1 Neurological 5 (7.4) 5 (25.0) 0.04 Lupus nephritis 15 (22.1) 7 (35.0) 0.37 APS diagnosis 9 (13.2) 5 (27.8) 0.15 Raynaud phenomenon 11 (16.2) 12 (60.0) <0.001 SD pattern at NVC, n (%) Capillary density, cap/mm (sd) Presence of megacapillaries, n (%) 7/68 (10.3) 8.86 (1.38) 6 (8.8) 6/9 (66.7) 7.48 (1.09) 6 (66.7) <0.001 0.004 <0.001 Acknowledgements Dr Marasco was supported by SLEuro for this project. *LC and MS are co-last authors. Disclosure of Interests None Declared.