The aim of this study was to evaluate health-related quality of life (HR-QOL) in patients with systemic sclerosis (SSc), to compare it with that of patients with rheumatoid arthritis (RA), and to correlate it with other parameters. HR-QOL was evaluated by the Short Form 36 (SF-36), SSc disease activity and severity by preliminary indexes recently proposed, disability by the Health Assessment Questionnaire (HAQ), and depressive symptoms by the Beck Depression Inventory. HR-QOL perception was not statistically different in patients with SSc and RA, except that patients with diffuse cutaneous involvement had worse scores in the general health and mental health dimensions than patients with RA (p=0.03). Compared with RA, patients with SSc tended to perceive less bodily pain (p=0.06) and have less disability (p=0.04) but to report higher depressive symptom scores (p=0.05). SSc patients' HR-QOL was associated with some disease severity scales (general, kidney and, less significantly, heart), but it was poorly correlated with the other evaluated disease activity and severity indexes. A strong correlation with disability and with depressive symptoms was observed. In conclusion, patients with SSc perceived a reduced HR-QOL similar to that of patients with RA. SF-36 may provide useful information in their evaluation.

Background Tocilizumab blocks pro-inflammatory activity of interleukin-6 (IL-6), involved in pathogenesis of pneumonia the most frequent cause of death in COVID-19 patients. Methods A multicenter, single-arm, hypothesis-driven trial was planned, according to a phase 2 design, to study the effect of tocilizumab on lethality rates at 14 and 30 days (co-primary endpoints, a priori expected rates being 20 and 35%, respectively). A further prospective cohort of patients, consecutively enrolled after the first cohort was accomplished, was used as a secondary validation dataset. The two cohorts were evaluated jointly in an exploratory multivariable logistic regression model to assess prognostic variables on survival. Results In the primary intention-to-treat (ITT) phase 2 population, 180/301 (59.8%) subjects received tocilizumab, and 67 deaths were observed overall. Lethality rates were equal to 18.4% (97.5% CI: 13.6–24.0, P = 0.52) and 22.4% (97.5% CI: 17.2–28.3, P < 0.001) at 14 and 30 days, respectively. Lethality rates were lower in the validation dataset, that included 920 patients. No signal of specific drug toxicity was reported. In the exploratory multivariable logistic regression analysis, older age and lower PaO2/FiO2 ratio negatively affected survival, while the concurrent use of steroids was associated with greater survival. A statistically significant interaction was found between tocilizumab and respiratory support, suggesting that tocilizumab might be more effective in patients not requiring mechanical respiratory support at baseline. Conclusions Tocilizumab reduced lethality rate at 30 days compared with null hypothesis, without significant toxicity. Possibly, this effect could be limited to patients not requiring mechanical respiratory support at baseline. Registration EudraCT (2020-001110-38); clinicaltrials.gov (NCT04317092).

Few data are available to assess the efficacy of rehabilitative interventions in systemic sclerosis (SSc). We refer here the results of an individualized rehabilitation program in 16 patients with SSc. In particular, when possible, the number of patients who achieved a minimal clinically important difference (MCID) was determined. Results were evaluated taking advantage of the development of validated questionnaires and tests to assess quality of life (QOL) and disability in SSc. At the end of a period of 4 months of observation, 69% and 62% of patients reported an improvement of the physical and mental components of the SF-36 higher than the MCID (as established in other rheumatic conditions). Analogously, an improvement of the impact of respiratory disease on patients' QOL, as assessed by the Saint George's Respiratory Questionnaire, was perceived by 67% of them. These results might be explained by better exercise tolerance, which was suggested by the significant reduction of the heart rate and of a visual analogue scale for dyspnoea at the end of the 6-min walking test. Finally, a statistically significant improvement of hand mobility, as assessed by the hand mobility in scleroderma test was obtained. This study suggests that a significant proportion of patients with SSc experience an improvement in their perception of QOL, a better exercise tolerance, and a better hand mobility after a rehabilitation program consisting by a 2-week period of daily individual 30-min sessions as outpatient, followed by at-home exercise program.

To verify the neuropsychological development in the offspring of patients with systemic lupus erythematosus (SLE), 47 children (23 male and 24 female) from affected women were studied. The tests applied were related to the children's ages: Griffiths scale up to four years, WPPSI and metaphonological tests (MP, evaluating the phonological consciousness) from four to six years of age, WISC-R test and Rey test (evaluating the visual-space abilities) from six years onwards; finally, specific tests for the diagnosis of learning disabilities (LD) between the ages of seven and 13. Intelligence levels were always normal (mean IQ score 106.32; median 104; SD 9.05). Three out of eight examined children failed MP, therefore may develop LD and will need further evaluation later. Fourteen children were specifically studied for LD and three reported scores lower than normal, but only two (who were brothers) were defined dyslexic. Antiphospholipid antibodies (aPL) were positive in the mothers of the three children with impaired LD tests. Other maternal autoantibodies or drugs administered during pregnancy did not seem to be related to LD. In conclusion, maternal SLE does not impair intelligence levels, but may increase the occurrence of LD particularly in male children (2/8 males examined, 25%). Both maternal aPL and genetic background may have pathogenetic implications.