AB0629 VASCULAR MANIFESTATIONS OF SYSTEMIC SCLEROSIS: SIMILAR IN PATHOGENESIS, DIFFERENT IN FREQUENCY

Abstract

Background:Pulmonary arterial hypertension (PAH) is one of the main manifestations of vascular involvement in systemic sclerosis (SSc). The association of PAH with Raynaud’s phenomenon (RP) and digital ischemic disorders is assumed.Objectives:The aim of the study to detect of the possible relationship of pathogenetically similar processes and the predictor role in the early diagnosis of PAH and digital ischemic disorders by the nailfold videocapillaroscopy (NVC).Methods:111 patients with SSc (51 patients with PAH (SSc-PAH) and 60 patients without PAH) include in this study. In all patients, the diagnosis of SSc was validated according to the 2013 ACR-EULAR criteria. PAH was diagnosed by right heart catheterization. NVC was performed in all recruited subjects. Capillary quantitative parameters (loops length and width, capillary density, neoangiogenesis) were evaluated and a semi-quantitative scoring was used (specific patterns - early, active and late) to define microvascular alterations. The test evaluated the presence of capillaroscopic changes in the nailfold bed on 2-5 fingers of both hands. The normal capillaroscopic pattern was characterized by the presence of 7-11 capillaries in the form of hairpins per 1 mm. Pathological patterns were characterized by morphological and structural changes, such as expanded and giant capillaries, hemorrhages, avascular fields, neoangiogenesis. The capillaroscopy pattern (normal, non-specific, early/active/late) was determined qualitatively. Decreased capillary density, dilated, giant or branched capillaries, microhemorrhages were evaluated semi-quantitatively.Results:RP was detected in 100% of cases in both groups. In the analysis of capillaroscopic patterns in both groups, the early and late scleroderma types of changes prevailed, but no significant differences were noted. Typical scleroderma patterns were found in 51 patients (100%) with SSc-PAH. In 3 patient with SSc without PAH, the abnormalities were regarded as non-specific. The NVC pattern was detected to be early in 8 patients with SSc-PAH and in 11 with SSc without PAH. The NVC pattern was found to be active in 16 patients with SSc-PAH and in 18 with SSc without PAH. The NVC pattern was detected to be late in 27 patients with SSc-PAH and in 28 with SSc without PAH. In addition to RP, the development of digital ulcers was noted with equal frequency in history (25 patients with SSc-PAH and 32 with SSc without PAH). Also, the time to their appearance from the first symptom of SSc was the same (56 (16; 84) months and 44 (23; 72) months, respectively). Severe forms of digital ischemic disorders were observed rarely and with the same frequency in the studied groups. Ischemia in 2 patients with SSc-PAH and in 5 patients with SSc without PAH, gangrene in 2 patients only in the SSc group without PAH, amputation in 1 of each group.Conclusion:In the course of the study, it was not possible to identify differences between the NVC patterns, the frequency and severity of digital ischemic disorders in the compared groups. That fact does not allow using the NVC as an early diagnosis of PAH in SSc. However, the NVC can help predict the development of digital ischemic disorders.