Sclero-Gyn: gynecological health and ultrasound evaluation among fertile women affected by systemic sclerosis; an observational study in a reference center.

Introduction. Systemic sclerosis (SSc) is a rare, immune-mediated and progressive multisystemic connective tissue disease. It is characterized by vasculopathy and abnormal collagen deposition, culminating in fibrosis of the skin and internal organs. SSc is predominantly observed in female, most prevalent in the third to fifth decade of life. The disease manifests itself in nearly 50% of patients during their reproductive years. SSc could affect various dimensions of women’s lives, including uro-gynecological health, reproduction, and sexuality. Although endometriosis remains an enigmatic condition with under-explored aetiology, immune system alterations have been proposed as a contributing factor to the development of ectopic endometrial tissue; however, there are no data on the incidence of autoimmune gynecological conditions such as endometriosis in patients affected by SSc. SSc is a condition recognized for its impact on blood vessels, specifically renal vessels, and may lead to severe complications such as a scleroderma renal crisis. Despite the similar capacity of the uterine and renal vessels, ultrasound evaluation and Doppler velocimetry of the uterine arteries have not yet been explored. The aim of the study. The purpose of this study is to provide a comprehensive report on the gynecological health of SSc-affected fertile women both clinically and ultrasoundly. Materials and Methods. The present study is an observational, cross-sectional, monocentric study performed in the scleroderma Unit of the University Hospital of Modena in collaboration with the Department of Medical and Surgical Sciences for Mothers, Children and Adults of the same institution. The study duration was 9 months. This study was conducted in two distinct phases. Initial phase consisted of a case-control study during which four validated questionnaires (SF-12, MEDI-Q, FSFI, USP) were administered to assess gynecological health, sexual well-being, and quality of life in both patients with systemic sclerosis and healthy controls (HC). The second phase involved a cross-sectional section, referring to a comprehensive pelvic gynecological and ultrasound evaluation exclusively in patients diagnosed with systemic sclerosis. Inclusion criteria were the diagnosis of SSc according to the 2013 ACR/EULAR criteria, adult age (≥18 years), fertile age, non-pregnant and non-breastfeeding. The age-matched HC were enrolled by routine controls in the gynecological clinic. Results. 29 Caucasian fertile patients with SSc and 33 HC gave their consent and were finally enrolled in the study. The mean age was 39.57 years, with a duration of the disease of 5.7 years. Half of the patients (53%) were diagnosed with lcSSc. The mean modified Rodnan skin score (mRSS) was 8 ±8.26, and 45% of the participants exhibited positivity for Scl/70. Scores for MEDI-Q, the FSFI full-scale score (with a cutoff of <26.5 indicating sexual dysfunction), and SF-12 were observed to be worse in patients with SSc. Furthermore, 10% of the patients reported a diagnosis of polycystic ovary syndrome (PCOS), while 17% were affected by endometriosis. Notably, 14% of the patients demonstrated at least one uterine artery with increased resistance to blood flow. Conclusions. Despite the limitations of the study, this well-selected sample for a unique referral center is considerable. The results highlight a high prevalence of gynecological, menstrual and sexual dysfunctions in patients with SSc, with a significant impact on quality of life. In our sample, the incidence of endometriosis is 17%, compared to 10% among fertile Italian women. This suggests that the incidence may be higher in SSc patients; but it should be taken with caution given the under-diagnosis of endometriosis. In our study, resistance in the uterine arteries exceeded 95 ° in 14% of the patients. To our knowledge, this is the first study to demonstrate the possible involvement of the uterine arteries by disease.