(1) Background: Systemic sclerosis (SSc) is characterized by significant fatigue, causing diminished quality of life (QoL). The aim of this study was to examine fatigue levels and their associations with clinical factors and determine the minimal clinically important difference (MCID) value for the Functional Assessment of Chronic Illness Therapy Fatigue Scale (FACIT-FS). (2) Methods: A total of 160 SSc patients and 62 individuals without SSc were followed-up over a 12-month period by measuring the FACIT-FS and the Visual Analogue Scale and the Short Form 36 Vitality Score analyzing changes in exhaustion. (3) Results: Fatigue was strongly correlated with HRQoL, level of pain, emotional disorders, physical capability and functionality. The MCID values for FACIT-FS were calculated as −3 for deterioration and +4 for improvement after a 12-month follow-up. The predictors of improvement of fatigue from baseline parameters were the significant disease activity, the patients’ poorer functionality and the short disease duration. Patients with scleroderma-related interstitial lung disease at baseline had approximately tripled risks for worsening fatigue. The independent influential factors regarding the changing of FACIT-FS were improving or worsening in the same direction in reference to physical condition, gastrointestinal and emotional factors. (4) Conclusions: Fatigue is a multi-dimensional symptom, which is strongly correlated to HRQoL. MCID values of FACIT-FS can be useful tools in monitoring the changes of HRQoL in clinical trials and in daily practice among patients with SSc.

Introduction: Nailfold video capillaroscopy (NVC) is a useful tool for measuring capillary density (CD) and capillary morphology parameters and is mainly used in systemic sclerosis (SSc). Objective: We aimed to assess the prevalence of an SSc pattern and CD in different connective tissue diseases (CTDs). Methods: NVC was performed on 268 patients with CTDs. Control groups consisted of 104 healthy volunteers (HVs) and 36 primary Raynaud’s patients (PRPs). Results: Decreased CD was more prevalent in SSc, systemic lupus erythematosus (SLE), inflammatory myopathies (IIM), and overlap CTD patients compared with both controls. Average CD, the prevalence of decreased CD, and the prevalence of an SSc pattern did not differ significantly between SSc patients with (n = 39) and without (n = 50) overlap syndrome. An SSc pattern was significantly more prevalent in SLE (23%), SSc (82%), IIM (35%), and rheumatoid arthritis (17%) compared with both control groups. The prevalence of an elevated microangiopathy evaluation score (MES) was significantly higher in SLE, SSc, and IIM than in the HVs. Conclusion: The presence of another CTD in SSc did not influence CD or morphology. An SSc pattern may also be present in CTDs other than SSc. The MES is a useful instrument to distinguish between patients with CTDs and controls.

B cell activation is an early event in the development of systemic sclerosis (SSc). The classical activation of B cells downstream of the B-cell receptor (BCR) involves the phosphatidylinositol-3 kinase (PI3K) pathway that integrates the effects of multiple co-stimulatory receptors. Our analysis of PI3K pathway associated molecules in peripheral blood B cells of early diffuse cutaneous SSc (dcSSc) patients showed altered mRNA expression of Toll-like receptor (TLR) homolog CD180, TLR4, complement component 3, IL-4 receptor and secreted phosphoprotein 1 (SPP1). Parallel to this, we found elevated basal SPP1 secretion in dcSSc B cells, but, with BCR + IL-4 receptor co-stimulation, we could not induce further secretion. CD180 stimulation alone resulted in NF-κB activation in more B cells than CD180 + BCR co-stimulation both in dcSSc and healthy control (HC), but the co-engagement increased the phosphorylation of NF-κB only in dcSSc B cells. Additionally, in contrast with HC B cells, the lower basal production of IL-10 by dcSSc B cells could not be elevated with CD180 stimulation. Furthermore, activation via CD180 increased the percentage of CD86+ switched memory (CD27+IgD−) B cells in dcSSc compared to HC. Our results suggest that alternative B cell activation and CD180 dysfunction cause imbalance of regulatory mechanisms in dcSSc B cells.

Background: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and visible skin changes that often result in body image dissatisfaction and impaired quality of life. Despite this, appearance-related concerns remain under-investigated in SSc. The Brief Satisfaction with Appearance Scale (Brief-SWAP) is a concise, validated instrument designed to assess body image dissatisfaction and social discomfort in individuals with visible disfigurement due to SSc. Objective: To adapt and validate the Brief-SWAP scale into Hungarian using internationally accepted methodology. Methods: Sixty patients with systemic sclerosis (53 women, 7 men; mean age 57.7 ± 13.5 years; 51.7% diffuse cutaneous SSc) and 31 age- and sex-matched healthy controls (mean age 57.9 ± 14.9 years) participated in the study. The adaptation process followed the OMERACT filter methodology, including forward-backward translation and cultural adaptation. Psychometric evaluation included structural validity (via principal component analysis) reliability (internal consistency and test-retest reliability), construct validity (convergent and discriminant validity) and content validity. Results: Structural validity confirmed a two subdomains model consistent with the original structure: dissatisfaction with appearance and social discomfort. Reliability was high: internal consistency was acceptable (Cronbach’s alpha= 0.716; p < 0.001) and test-retest reliability was strong (ICC= 0.828). Regarding construct validity, discriminant validity was demonstrated by significantly higher Brief-SWAP scores in SSc patients versus controls (median 13.0 [IQR 6.0; 18.0] vs. 3.0 [1.0; 9.0]; p < 0.001) with no significant difference between limited and diffuse cutaneous SSc subgroups. Convergent validity showed moderate correlations with depression scales and SF-36 components. Content validity was confirmed, with no floor or ceiling effects observed. Conclusions: The Hungarian version of the Brief-SWAP is a valid and reliable instrument for assessing body image dissatisfaction and its psychosocial impact in patients with SSc. Its brevity and ease of use make it suitable for clinical and research applications focused on health-related quality of life.

Objectives: To investigate the impact of gastrointestinal involvement in systemic sclerosis affecting the quality of life and systemic sclerosis-related disability using patient-reported outcome measurements (PROMs). Methods: Data from 160 consecutive systemic sclerosis patients were collected, including clinical characteristics and self-assessment questionnaires. The severity of gastrointestinal involvement was determined by the University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA-GIT 2.0): patients were classified as having “none-to-mild” symptoms (total score = 0.00–0.49: Group A), “moderate” symptoms (Group B), and “severe-to-very severe” symptoms (Group C). All investigations were repeated following 1 year to check the reliability (n = 149). Results: Quality of life (EuroQol-5 Dimension anxiety/depression, Physical Component Summary, and Mental Component Summary of Short-Form Health Survey-36) and disability questionnaires (Health Assessment Questionnaire Disability Index, Scleroderma Health Assessment Questionnaire), Scleroderma Impact of Disease showed moderate significant correlation with UCLA-GIT 2.0 (rho: 0.400, −0.484, −0.468, 0.400, 0.436, 0.646, respectively). These correlations remained significant when tested in the two main subgroups (limited and diffuse cutaneous systemic sclerosis). Disease duration and UCLA-GIT 2.0 score did not show any correlation (rho: .055, p = 0.488). The average gastrointestinal involvement complaint of early systemic sclerosis (disease duration < 3 years, n = 24) subgroup measured by the UCLA-GIT 2.0 was not different from the rest of the cases (0.13 (0.03-0.46) vs 0.22 (0.08-0.45), p = 0.280). All investigated PROMs regarding quality of life and disability showed worse results in Group B when compared with patients in Group A. Similarly, patients in Group C have worse results compared to Group B. These correlations with the UCLA-GIT 2.0 score were confirmed during the 1-year follow-up. Conclusion: Moderate/severe gastrointestinal involvement symptoms based on the UCLA-GIT 2.0 are associated with worse quality of life and disability. This correlation holds for both systemic sclerosis subgroups and independent of disease duration: moderate/severe gastrointestinal involvement complaints develop early in systemic sclerosis parallel with early onset of decreased quality of life and disability.