Background:Systemic sclerosis is a rare disease determining a damage to the connective tissue and, consequently, an involvement of several organs. Besides the damage of the connective tissue, preminent is also the small vessels injury, detectable by videocapillaroscopy. Some authors report that the vascular damage may be also responsible of a cardiovascular impairment as cardiac autonomic disease (CAN) and heart rate variability [1].Objectives:Our study aims to assess the presence and entity of CAN in patients with systemic sclerosis (SSc).Methods:This is a pilot prospective cohort study. We enrolled 28 patients in a period of six months, from May 2019 to November 2019, afferent to the outpatient clinic of internal medicine and immunology of the Primo Policlinico of Naples, with definite SSc diagnosis in absence of other comorbidities. All patients underwent diagnostic tests for autonomic cardiac neuropathy (NAC) and videocapillaroscopy. In particular, four test were performed to search for the presence of NAC: orthostatic hypotension, deep breathing, lying to standing and Valsalva maneuver. Each test was corrected for age and diagnosis was made in the case at least two tests resulted positive. Primary endpoint of the study was the assessment of the prevalence of autonomic cardiac neuropathy in the study population.Results:Our cohort was mainly characterized by females (92.9%), with a median age of 58.5 years [IQR: 49-64.8 yrs.] and a median duration of the disease of 4 years [IQR 2-13 yrs.]. The observed prevalence of NAC was equal to the 46.4% (13 cases). In addition, we evaluated the potential association of NAC with age, duration of disease, gastrointestinal dysmotility, sicca syndrome, cutaneous involvement and type of videocapillaroscopy pattern, from which no statistically significant result emerged. Hence, a further analysis, by using a time-dependent Cox regression model (with the duration of disease as time covariate), was performed on the same variables. From this model a significant association emerged in particular between the presence of NAC and the active videocapillaroscopy pattern (OR 6.23; 95% CI: 1.058-36.71, p=0.043).Conclusion:Though current data in the literature on this topic are poor, cardiac autonomic neuropathy is among the clinical manifestations of SSc. In our study population, though the limited sample size, we observed a high percentage of patients with autonomic cardiac neuropathy, which seems much more frequent with the increase in the duration of disease and based on the type of videocapillaroscopy pattern.References:[1]Ferri C, Emdin M, Giuggioli D, Carpeggiani C, Maielli M, Varga A, Michelassi C, Pasero G, L’Abbate A. Autonomic dysfunction in systemic sclerosis: time and frequency domain 24 hour heart rate variability analysis. Br J Rheumatol. 1997[2]Adler BL, Russell JW, Hummers LK, McMahan ZH. Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire. J Rheumatol. 2018 Aug;45(8):1145-1152. doi: 10.3899/jrheum.170868. Epub 2018 JunDisclosure of Interests:None declared
Assessment of heart rate variability and tone of the autonomic nervous system in puberty-aged children with undifferentiated connective tissue dysplasia
