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Rituximab (RTX) has been used for the treatment of systemic sclerosis (SSс) for a long time and has shown good efficacy for skin fibrosis and interstitial lung disease (ILD). However, data on tolerability and long-term adverse events (AEs) during RTX therapy in SSc are insufficient. The objective of this study was to assess the tolerability and safety of RTX in patients with SSс in a long-term prospective follow-up. Our open-label prospective study included 151 SSс patients who received at least one RTX infusion. The mean age of the patients was 47.9 ± 13.4 years; the majority of them were women (83%). The mean disease duration was 6.4 ± 5.8 years. The mean follow-up period after the first RTX infusion was 5.6 ± 2.6 years (845.6 patient-years (PY)). All patients received RTX in addition to ongoing therapy with prednisone and/or immunosuppressants. AEs were assessed and recorded by a doctor in the hospital immediately after RTX infusion and then by patient's reported outcome during the observation period. All causes of death were considered, regardless of treatment. A total of 85 AEs (56%) were registered, the overall incidence of AEs was 10/100 PY (95% confidence interval (CI) 8-12). The highest frequency of all AEs was observed in the first 2-6 months after the first course of RTX, however, these were mainly mild and moderate AEs (71%). The most frequent AEs were infections, they were observed in 40% of cases, with no serious opportunistic infections reported. The overall incidence of all infections was 7.1/100 PY (95% CI 5.5-9), serious infections-1.5/100 PY (95% CI 0.9-2.6). Infusion reactions occurred in 8% of patients. Other AEs were noted in 3% (0.6/100 PY, 95% CI 0.3-1.4). The overall incidence of serious AEs was 18%-3.2/100 PY (95% CI 2.2-4.6). There was a significant decrease of the immunoglobulin G (Ig G) during follow-up; however, its average values remained within normal limits. There were 17 deaths (11%) (2/100 PY, 95% CI 1.3-3.2). In most cases, patients died from the progression of the major organ failure, which arose before RTX treatment. In our study, the safety profile of RTX in SSс was assessed as favorable. It was similar to the AE profile in other autoimmune diseases treated with RTX. With an increase in the cumulative dose of RTX, no increase in AEs was observed. The mortality is comparable to the other severe autoimmune diseases in observational studies. Monitoring of IgG may be useful for patients with SSс on RTX therapy for early detection of the risk of developing infectious complications. RTX could be considered as a relatively safe drug for the complex therapy of SSс when standard therapy is ineffective or impossible., (© 2024. Pleiades Publishing, Ltd.)

The aim of our study was to assess the relationship between the changes of antinuclear autoantibodies (ANAs) and autoantibodies to topoisomerase 1 (anti-Topo 1) in systemic sclerosis (SSs) patients on rituximab (RTX) therapy. The prospective study included 88 patients (73 women) with a mean age of 47 (17–71) years. The mean disease duration was 5.9 ± 4.8 years. The mean follow-up period was more than 2 years (27 (12–42) months). We documented a statistically significant change in skin score, the disease activity index, improvement of pulmonary function and reduction of mean dose of prednisolone after RTX treatment. There was a significant decrease in the number of patients with high levels of ANA and overall decrease of the ANA and anti-Topo 1 levels. A moderate positive statistically significant correlation was found between ANA and anti-Topo 1 (r = 0.403). In the group of patients positive for anti-Topo 1 there were a more pronounced depletion of B lymphocytes, significantly higher increase in forced vital capacity and diffusion capacity, decrease in the disease activity index, compared with patients negative for anti-Topo 1. We observed the decline in the level of ANA and anti-Topo 1 in SSc patients after RTX therapy, and it was correlated by an improvement of the main outcome parameters of the disease. Therefore, anti-Topo 1 positivity could be considered as a predictor of a better response to RTX treatment, especially in SSc patients with hyperproduction of anti-Topo 1. [ABSTRACT FROM AUTHOR]

Objectives: Osteoporosis is prevalent in patients with systemic sclerosis (SSc). Updated evidence is required to complement the previous systematic review on this topic to provide best practices. This systematic review and meta-analysis aimed to quantitatively synthesize data from studies concerning the prevalence and risk factors for osteoporosis among patients with SSc. Methods: We searched PubMed, EMBASE, Web of Science, and ScienceDirect databases for potential studies published from inception to May 31, 2022. Eligibility screening, data extraction, and quality assessment of the retrieved articles were conducted independently by two reviewers. Then meta-analyses were performed to determine osteoporosis prevalence and risk factors in patients with SSc. Meta-regression analysis was conducted to explore the sources of heterogeneity. Results: The pooled prevalence of osteoporosis in patients with SSc was 27% (95% CI, 24–31), with moderate heterogeneity (I2 = 61.6%). Meta-regression revealed no significant difference among all variables. And the presence of SSc increased the likelihood of having osteoporosis (OR = 3.05, 95% CI, 2.32–4.01) compared to controls. These significant risk factors for osteoporosis in SSc patients were age > 50 years (OR = 2.94, 95% CI, 1.52–5.68), menopause (OR = 3.90; 95% CI, 1.94–7.84), aging (MD = 8.40; 95% CI,6.10–10.71) and longer disease duration (MD = 4.78; 95% CI,1.83–7.73). However, female (OR = 1.45; 95% CI, 0.75–2.77), pulmonary arterial hypertension (OR = 0.50; 95% CI, 0.17–1.54), and diffuse cutaneous SSc (OR = 1.05; 95% CI, 0.75–1.48) were not significant risk factors for osteoporosis in SSc patients. Conclusions: Osteoporosis was highly prevalent in patients with SSc, and the prevalence seemed to be high and similar in many countries. The age > 50 years, menopause, aging, and longer disease duration were identified as risk factors for osteoporosis in patients with SSc. Key Points • The pooled prevalence of osteoporosis in patients with SSc was 27% based on 22 included studies. • SSc patients had an increased prevalence and risk of osteoporosis as compared to the healthy control population. • Age > 50 years, menopause, aging, and longer disease duration might be associated with osteoporosis in SSc. [ABSTRACT FROM AUTHOR]