Impact of systemic sclerosis on outcomes of patients hospitalized for influenza: Evidence from the US nationwide inpatient sample.

Liver involvement, particularly autoimmune hepatitis (AIH) overlap, is a rare but clinically important manifestation of systemic sclerosis (SSc). This study aimed to assess the prevalence, clinical characteristics, and diagnostic utility of laboratory parameters for identifying AIH in a cohort of patients with SSc. We retrospectively analyzed 111 patients with SSc. Clinical characteristics, autoantibody profiles, and laboratory parameters were compared between patients with and without AIH. AIH diagnosis was confirmed by liver biopsy in all cases. Given the small number of events, Firth's penalized likelihood logistic regression was applied to identify independent risk factors. The diagnostic performance of alanine aminotransferase (ALT), aspartate aminotransferase (AST), and serum IgG levels was evaluated using receiver operating characteristic (ROC) curve analysis. Prior methotrexate (MTX) exposure was recorded, and the potential confounding effect of MTX-induced liver enzyme elevations was assessed. Autoimmune hepatitis (AIH) was identified in 8 of 111 patients (7.2%). All AIH-positive patients showed interface hepatitis on liver biopsy. There were no significant differences between AIH-positive and AIH-negative patients regarding age, systemic sclerosis subtype, or presence of interstitial lung disease (all p > 0.05). Firth's penalized logistic regression indicated that diffuse cutaneous SSc, anti-Scl-70 positivity, and interstitial lung disease were not independent predictors of AIH overlap. In receiver operating characteristic analysis, alanine aminotransferase (ALT) demonstrated the highest diagnostic performance (AUC 0.88, 95% CI 0.76-0.99; p < 0.001), with a sensitivity of 87.5% and specificity of 85.4% at a cut-off > 34.5 U/L. Aspartate aminotransferase (AST) (AUC 0.86) and serum IgG (AUC 0.74) also showed significant but lower discriminatory ability. ALT retained high specificity for AIH even among patients with prior MTX exposure, supporting its utility as a non-invasive screening tool in SSc. Autoimmune hepatitis is a rare overlap syndrome in systemic sclerosis that occurs independently of clinical phenotype. Routine monitoring of liver transaminases, particularly ALT, provides a reliable non-invasive screening tool. Clinicians should consider ALT elevations > 34.5 U/L as a trigger for further AIH evaluation, even in patients with prior MTX exposure. Key Points •Autoimmune hepatitis (AIH) represents a clinically significant overlap in systemic sclerosis (SSc), with a prevalence of 7.2% in this cohort, indicating that it may be more common than previously reported when systematic screening is implemented. Biochemical screening rather than biopsy-only evaluation improves detection. •The development of AIH in SSc patients appears to be independent of disease subtype (limited vs. diffuse), specific autoantibody profiles (Anti-Scl70, ACA), or major organ involvement, such as interstitial lung disease. •An ALT threshold of > 34.5 U/L provides a practical "red flag" for clinicians, offering high sensitivity and specificity to prompt further diagnostic evaluation, including liver-specific autoantibody testing and liver biopsy when indicated. This threshold should be interpreted cautiously in patients with prior methotrexate (MTX) exposure, as mild ALT elevations may also reflect drug-related hepatotoxicity. •An ALT threshold of > 34.5 U/L provides a practical "red flag" for clinicians, offering high sensitivity and specificity to prompt further diagnostic evaluation, including liver-specific autoantibody testing and liver biopsy when indicated. •Because AIH can develop across the full spectrum of SSc regardless of systemic disease severity, routine monitoring of transaminases is essential for early detection and timely initiation of immunosuppressive therapy.

Research on the mechanism of Carthamus tinctorius L. against skin fibrosis in systemic sclerosis based on serum pharmacochemistry, network pharmacology, and experimental validation.

Systemic sclerosis (SSc) is characterized by early and persistent vascular injury, immune dysregulation, and fibrosis, with a growing recognition of an excess thrombotic burden that cannot be fully explained by traditional cardiovascular risk factors. Increasing experimental and clinical evidence positions platelets as mediators at the interface of vasculopathy, inflammation, and coagulation in systemic sclerosis. This review addresses emerging platelet-driven mechanisms that extend platelet function beyond haemostasis and highlight their role as intravascular messengers capable of propagating damage across organs. Recent literature demonstrates that platelets in SSc exhibit a persistently activated and primed phenotype, driven by endothelial injury, aberrant platelet-collagen interactions, inflammation-mediated priming, and defective platelet clearance. Activated platelets interact dynamically with immune cells, particularly neutrophils, promoting neutrophil extracellular trap formation and immunothrombosis through pathways involving HMGB1, P-selectin-PSGL-1, and GPVI. Platelet-derived mediators, including CXCL4, serotonin, PDGF, and extracellular vesicles, enable the dissemination of inflammatory and profibrotic signals, thereby contributing to endothelial dysfunction, immune activation, and fibrotic remodelling in distant vascular beds. Clinical studies increasingly link platelet activation markers with distinct disease subsets and major organ complications. Collectively, these findings identify platelets as key orchestrators and conveyors of immunothrombosis and thromboinflammation in SSc. Improved understanding of platelet-driven signalling networks may inform risk stratification and support the development of targeted antithrombotic or immunomodulatory strategies, although robust evidence for disease-modifying antiplatelet interventions remains limited.

This study aimed to evaluate the global trends in systemic sclerosis (SSc)-related mortality by age, sex, and geographic region. SSc is a multisystem autoimmune disease characterized by tissue fibrosis, vascular dysfunction, and multi-organ involvement, which is associated with a high mortality risk. Using the World Health Organization Mortality Database, we examined trends in SSc-related crude mortality rates (SSc-CRs) and age-standardized mortality rates (SSc-ASMR) per 1,000,000 population from 2001 to 2023. Locally weighted regression was applied to visualize long-term patterns, and Joinpoint regression was used to assess the national trends from 2010 to 2023. Across 74 countries, 85,291 SSc-related deaths were reported, with 79.41% occurring in females. The SSc-CR steadily increased from 1.97 (95% confidence interval [CI]: 1.71-2.23) in 2001 to 2.34 (95% CI: 2.01-2.68) in 2023, while the SSc-ASMR decreased from 1.58 (95% CI: 1.42-1.74) to 1.29 (95% CI: 1.08-1.50), respectively. Regionally, mortality was the highest in the Western Pacific region and declined in the Americas and Europe, with temporal fluctuations. The SSc-ASMR was highest in countries with a middle sociodemographic index (SDI). While overall age-standardized mortality from SSc has declined in many regions, disparities persist. These results underscore the importance of sustaining research and enhancing disease awareness, as well as developing strategies to reduce mortality in high-risk populations and regions. Key Points • First global analysis ofmortality trends across 74 countries (2001-2023) • Age-standardized mortality declined globally, but crude mortality increased, with persistent female predominance • Findings highlight need for targeted strategies, early diagnosis, and improved care to reduce mortality.

Autologous hematopoietic stem cell transplantation (aHSCT) for systemic sclerosis (SSc) is an effective treatment strategy but carries a high treatment-related mortality (TRM). Consequently, patients with severe organ dysfunction have been excluded from previous randomized trials such as ASTIS. This study aimed to evaluate the feasibility and non-inferiority of a disease-manifestation-adapted chemotherapy protocol designed to mitigate toxicity to also treat patients who would have been ASTIS-ineligible. In this prospective, open-label, monocentric, phase II non-inferiority trial at the University Hospital Tübingen, Germany, patients with progressive SSc (disease duration≤6 years) were stratified by lung and cardiac involvement. Mobilization included 1500mg/m2 cyclophosphamide (CYC) for patients with inflammatory interstitial lung disease (iILD) versus 1000mg/m2 otherwise, both reduced compared to the ASTIS protocol. Conditioning comprised rabbit anti-thymocyte globulin (4×10mg/kg); patients without cardiac involvement additionally received CYC 4×50mg/kg. In those with cardiac involvement, CYC was reduced by 50% and thiotepa (2×5mg/kg) was added. Between 09/2012 and 07/2022, 35 patients were treated (no iILD/cardiac involvement: n=14; iILD only: n=3; cardiac only: n=12; both: n=6). Three-year overall survival (OS) was 77%, meeting non-inferiority compared to EBMT registry data (80%). TRM within 100 days was 11% (n=4) in the whole group. This adapted regimen showed comparable 3y-OS in patients with advanced organ involvement who would have been excluded from prior trials. While feasibility is demonstrated, aHSCT in this high-risk population remains associated with substantial risk, and efficacy cannot be definitively established. Further studies are warranted.

Anti-CD52 therapy of rheumatic diseases: Revisited in the era of immune reset.

Systemic sclerosis (SSc) is characterized by progressive dermal fibrosis and microvascular dysfunction, and no approved therapy reliably reverses established skin fibrosis or durably restores microvascular perfusion. Adipose-derived stem cells (ASCs) possess anti-fibrotic, immunomodulatory, and vascular-related parameters properties, but their therapeutic impact in a strictly therapeutic (rather than preventive) SSc-like setting remains incompletely defined. Bleomycin-induced systemic sclerosis model was induced in male C57BL/6 mice by daily subcutaneous bleomycin injections (100μg) into dorsal skin for 28days. On day 14, mice received a single intralesional injection of ASCs (1 × 105 cells) or vehicle. At day 28, cutaneous perfusion was measured by laser Doppler perfusion imaging, and dorsal skin was analyzed by histology, hydroxyproline assay, RT-qPCR, and immunohistochemistry for CD34, α-SMA, and TNF-α. To support mechanistic interpretation, TGF-β1-stimulated dermal fibroblasts were co-cultured with ASCs and fibrosis-related gene expression was assessed. Intralesional ASC administration significantly attenuated bleomycin-induced dermal fibrosis, reducing dermal thickness (244.0-163.5μm) and collagen area fraction (87.2-62.8%). Hydroxyproline content decreased from 0.187 to 0.121μg/mg tissue. ASC treatment also suppressed profibrotic and inflammatory transcripts (α-SMA ~ 3.99-fold, TGF-β1 ~ 6.07-fold, TNF-α ~ 7.48-fold, IL-6 ~ 2.36-fold vs. BLM + PBS) and increased vascular responses transcripts (VEGF ~ 2.65-fold, CD34 ~ 1.28-fold vs. BLM + PBS). ASC co-culture suppressed profibrotic activation of TGF-β1-stimulated fibroblasts, reducing profibrotic expression (α-SMA ~ 2.5-fold, TGF-β1 ~ 3.5 -fold, and COL1A1 ~ 2.7-fold). A single intralesional ASC injection alleviated established bleomycin-induced dermal fibrosis and was associated with vascular-related changes in fibrotic tissue. These effects may involve paracrine-mediated suppression of TGF-β1-driven fibroblast activation, supporting ASCs as a promising regenerative strategy for systemic sclerosis skin disease.

Rituximab is effective in both systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and cutaneous sclerosis. However, most studies report improvement in skin sclerosis as a secondary outcome, leaving a gap in the literature on rituximab's dermatological impact. To evaluate rituximab's effectiveness in reducing skin sclerosis. This retrospective study was conducted at a tertiary care centre in northern India. Records of patients with SSc from 2016 to 2023 were reviewed. Clinical assessments, investigations and standard treatment for end-organ damage were undertaken according to clinical presentation and the European Alliance of Associations for Rheumatology guidelines. Rituximab was administered in two doses of 1 g each, administered 2 weeks apart to patients with a baseline modified Rodnan skin score (mRSS) of ≥ 14 with or without ILD or a baseline mRSS of ≥ 7 and < 14 in the presence of ILD, as per a departmental protocol. Patients were divided into two groups: those receiving rituximab (the rituximab group) and patients who were not treated with rituximab (the non-rituximab group). Treatment response was evaluated based on change in mRSS and overall survival. Among 98 patients with SSc, 37 received rituximab and 61 were in the non-rituximab group. The rituximab group had a higher percentage of patients with diffuse skin sclerosis (DSS) [70% (26/37) vs. 30% (18/61)] and a higher mean baseline mRSS [20.1 (SD 9.4) vs. 11.5 (SD 8.0)]. There was a significantly greater mean percentage reduction in mRSS [47.9% (SD 27.4) vs. 31.2% (SD 31.4), P = 0.01] in the rituximab group compared with the non-rituximab group. Overall survival (Kaplan-Meier curve analysis) was 66% at 140 months in the rituximab group compared with 53% at 95 months in the non-rituximab group (P = 0.85). Patients with limited skin sclerosis (LSS) had better survival (78% at 100 months) compared with those with DSS (47% at 140 months, P = 0.05). On binomial logistic regression analysis, a greater percentage reduction in mRSS was the strongest predictor of survival (P = 0.04). Rituximab treatment resulted in a significant reduction in skin sclerosis even in patients with more severe baseline skin sclerosis, although it did not demonstrate any survival benefit.

Hyperuricemia influences several aspects of the immune system. It enhances cytokine production by monocytes and activates neutrophils and natural killer cells. Within the adaptive immune system, hyperuricemia enhances antigen presentation, skews T helper cell differentiation toward the Th17 lineage and may also activate B cells. Beyond its established role in the pathogenesis of gout, hyperuricemia may therefore contribute to other rheumatic diseases. In this review, we summarize current evidence on the role of hyperuricemia in osteoarthritis, psoriatic arthritis, axial spondylarthritis, rheumatoid arthritis, systemic sclerosis, primary Sjögren’s disease and systemic lupus erythematosus. Available data do not support a causal role for hyperuricemia in the disease onset of osteoarthritis or rheumatoid arthritis. In contrast, hyperuricemia is associated with the development of psoriatic arthritis and may be linked to a more severe disease course. Small, predominantly cross-sectional studies further suggest a potentially adverse role of hyperuricemia in systemic sclerosis, Sjögren’s disease, and systemic lupus erythematosus. Across several rheumatic diseases, hyperuricemia is associated with cardiovascular disease, renal dysfunction and interstitial lung disease. However, both mechanistic and causal evidence remain limited, underscoring the need for more studies.

Cardiovascular death is the second leading cause in systemic sclerosis (SSc), with coronary microvascular dysfunction (CMVD) playing a key role. PET-derived myocardial blood flow (MBF) and flow reserve (MFR) offer a validated, noninvasive way to assess CMVD. We studied the prevalence of impaired MFR in SSc and its association with vasodilator or immunomodulatory therapy. SSc patients who underwent dynamic 82-Rubidium PET myocardial perfusion imaging (MPI) at Yale New Haven Hospital (July 2016 to April 2025) were studied. Patients were matched 3:1 with controls without autoimmune rheumatologic disease based on age, sex, body mass index, and cardiovascular comorbidities. Abnormal MFR was defined as < 2.0. The cohort included 67 SSc patients (87% female, age : 61 ± 11 years, 21% diffuse cutaneous SSc, SSc duration : 12 ± 10 years) and 201 controls. Rest MBF was higher in SSc (1.14 [IQR 0.91-1.39] ml/g/min) vs controls (1.00 [IQR 0.78-1.25]; p= 0.01), while MFR was lower (2.17 [IQR 1.84-2.57] vs 2.44 [IQR 1.96-2.96]; p= 0.01). Stress MBF was similar (2.47 [IQR 1.99-2.82] vs 2.43 [IQR 1.91-3.04]; p= 0.85). Multivariable analysis showed mycophenolate mofetil (MMF) use linked to lower odds of abnormal MFR (OR 0.09 [95% CI 0.01-0.56]; p= 0.017), while calcium-channel blockers (OR 7.77 [1.93-42.53]; p= 0.008) and statins (OR 7.14 [1.86-36.87]; p= 0.008) increased odds. PET MPI reveals reduced MFR in SSc. Treatment associations, including MMF, should be interpreted cautiously given the retrospective design. PET-derived MFR may serve as a noninvasive marker of vascular involvement, warranting prospective validation.

Patients with systemic sclerosis (SSc) exhibit systemic and more pronounced micro- and macro-architectural bone damage compared to healthy subjects (HS). The extent of the microvascular damage in SSc may be associated with the severity of compromised systemic bone integrity. The aim of this study is to investigate and score the status of the hand bone mineral density (BMD) in patients with SSc, using a new dedicated hand software and to score the microvascular status of the same hands by using nailfold videocapillaroscopy (NVC). Bone mineral density (BMD /g/cm2) and bone mineral content (BMC /g) of left and right hand using a new hand dedicated software (enCore, GE Lunar Prodigy Bone Densitometer, BMD hand software, USA), as well total BMD of the skeleton (GE Lunar Prodigy Bone Densitometer), were measured in 32 SSc patients classified according to the 2013 ACR/EULAR criteria (mean age 61±14 years, 94% women, 47% (n=13) dcSSc and in 27 age-matched HS. Quality of peripheral microvascular involvement (NVC patterns) was evaluated via standardised NVC analysis including capillary number scoring. SSc organ involvement was evaluated according to the 2023 EULAR recommendations. Statistical analysis included non-parametric and multivariable regression analyses to explore the relationship between capillary density and hand bone status. A multiple regression analysis demonstrated that left- and right-hand BMD was significantly associated with capillary loss after adjustment for age, sex, BMI, osteoporosis history, grip strength, immunosuppressive therapy and bone-specific treatments (p=0.02 and p=0.03). Interestingly, BMD values were found positively correlated with the absolute number of capillaries per linear millimetre (left hand r=0.6893, p<0.001; right hand r=0.45, p=0.03). A significant negative correlation was also found between left hand BMD (r=-0.5752, p=0.001) with the presence of "late" NVC scleroderma pattern. A significant negative correlation was finally observed between left hand BMD and the presence of dcSSc (r=-0.3836, p=0.036) and the modified Rodnan skin score (r=-0.5811, p=0.002). Moreover, SSc patients exhibited significantly lower hand BMD compared to HS even adjusted for age, sex, BMI and history of osteoporosis. For the first time, hand local bone status was found significantly associated with hand/fingers NVC microvascular damage in SSc patients, emphasising the effects of capillary loss/local hypoxia on the observed bone loss. At the same time, the results of the regression model reinforced the role of capillary loss as a potential predictor of hand bone quality in SSc patients.

Cardiac involvement is one of the severe manifestations of systemic sclerosis (SSc), ranging from subclinical forms to life-threatening conditions, and is characterized by poor prognosis and high mortality. Cardiopathy in SSc may be primary or secondary, associated with comorbid conditions. Therefore, timely diagnosis of primary cardiac involvement is of particular importance, including the assessment of serum biomarkers.This review discusses laboratory biomarkers for the detection of primary cardiac involvement in SSc, their role in early diagnosis, and their value in predicting disease course.

Systemic sclerosis (SSc) is characterized by fibrosis and vasculopathy affecting the skin and internal organs, leading to multiorgan dysfunction. Injury of microvascular endothelial cells (ECs) in SSc impairs blood flow and causes tissue ischemia, leading to vascular complications such as Raynaud's, digital ulcers, and pulmonary hypertension (PH). PH in SSc presents as group 1 pulmonary arterial hypertension or as group 3 PH related to hypoxia and interstitial lung disease (ILD), both major causes of mortality. Analysis of multiome data from SSc ILD-PH lungs inferred transcription factors regulating EC phenotype, including FOSL2. Overexpression of FOSL2 in transgenic mice (Fosl2tg) leads to vascular changes mirroring human SSc-PH, such as intimal thickening and fibrosis. scRNA-Seq analysis of altered EC gene expression in Fosl2tg mice showed strong overlap with altered EC gene expression in SSc-ILD-PH. Overlapping as well as discrete EC gene expression in Sugen/hypoxia- and hypoxia-treated mice suggested that FOSL2 regulates both hypoxia-dependent and -independent pathways in Fosl2tg mice and SSc-ILD-PH. A deep learning model, ChromBPNet, inferred increased AP-1 binding at base pair resolution in SSc-ILD-PH ECs, and binding to the same motifs was found upon FOSL2 overexpression in primary vascular ECs, highlighting FOSL2's key role in driving the pathological changes seen in SSc-ILD-PH.

Objective: to assess the prevalence of systemic lupus erythematosus (SLE) in the Russian population of patients with reproductive health problems (RHP). Material and methods. Patients were enrolled in 2023–2024 at 15 clinical centers in 5 federal districts of the Russian Federation. Women referred by a gynecologist, reproductive specialist, or general practitioner to a rheumatologist to rule out a systemic autoimmune rheumatic disease (SARD) were included. The study comprised two visits: the first screening visit and the second (final, 2–6 weeks later) visit, during which SARD verification was performed. Descriptive statistics methods were used for data analysis. The frequency of features in two independent study groups was compared using χ2 (Pearson’s test), odds ratios with 95% confidence intervals. The association between antibody levels and clinical manifestations was assessed by constructing a regression model. Results and discussion. Of 631 women examined, 612 (97.0%) met the inclusion criteria; 19 women were excluded because they did not have RHP (infertility or pregnancy loss) and/or signs of SARD. Antinuclear factor (ANF) was determined in 590 of 612 women. ANF positivity was detected in 238 (40.3%) of 590 patients. Of these 238 women, antinuclear antibodies were detected in 71, and decreased complement component levels were found in 55 (C3 in 28 and C4 in 27). SARD was diagnosed in 71 (11.6%) cases: SLE in 27 (4.4%) (definite in 23 and probable/incomplete in 4), mixed connective tissue disease was newly diagnosed in 36 (5.9%), Sjцgren’s disease in 4 (0.7%), and systemic sclerosis in 4 (0.7%). Among ANF-positive patients with RHP, 167 (70.2%) of 238 had no SARD. Conclusion. In this multicenter pilot study, the frequency of SLE in the Russian population among women with RHP was 4.4% (27 of 612 women), and the overall frequency of SARD was 11.6% (71 of 612). ANF positivity occurred more than three times (40.3%) more often than newly diagnosed SARD cases, indicating the need for further studies to clarify the reasons for ANF occurrence in women of childbearing age with RHP and for their longitudinal follow-up.

In recent years, significant progress has been achieved in the study of systemic sclerosis (SSc). The treatment of SSc includes conventional synthetic disease-modifying antirheumatic drugs, biologic agents, including anti-B-cell drugs and interleukin-6 inhibitors, as well as antifibrotic agents. Since the last publication of the EULAR recommendations for the treatment of SSc in 2017, changes have occurred; therefore, an update was prepared in which the treatment of the full spectrum of SSc manifestations is considered more broadly. In the updated document 22 recommendations were formulated, and 8 therapeutic domains were identified. The article presents the EULAR 2023 recommendations for the treatment of SSc, as well as promising directions for scientific research.

Introduction Systemic sclerosis (SSc) is a complex, autoimmune disease associated with reduced bone density and increased fragility. The study aims to investigate the molecular background of early bone alterations in SSc using dual X-ray absorptiometry (DXA)-derived bone mineral density (BMD) parameters and their associations with osteoimmunological biomarkers. Material and Methods The prospective cross-sectional study included 40 women with SSc of relatively short disease duration (≤ 7 years from diagnosis) and 45 age-, sex- and bone status-matched non-immune controls at the Department of Rheumatology and Immunology, Clinical Centre, University of Debrecen, Hungary. Bone status was assessed by DXA at the lumbar spine and femoral neck. Routine bone and mineral metabolism markers were measured, as well as a targeted panel of osteoimmunological mediators (osteoprotegerin [OPG], osteopontin [OPN], platelet-derived growth factor-BB [PDGF-BB], tartrate-resistance acid-phosphatase [ACP5], receptor activator of NF-κB ligand [RANKL], tumour necrosis factor [TNF], interleukin-6 [IL-6], IL-1β, Dickkopf-1 [DKK-1]), which were quantified by multiplex immunoassay. Ten-year probabilities of major osteoporotic fracture and hip fracture were calculated using the FRAX® algorithm. Results The DXA-defined bone status distribution did not differ between SSc patients and controls, yet FRAX estimates were numerically higher in SSc. Serum OC levels were significantly lower, while 25-hydroxy vitamin D levels were significantly higher in SSc compared to non-SSc group, reflecting more frequent supplementation (Mann-Whitney U test, p = 0,05). Whereas SSc exhibited a distinct osteoimmune serum profile, characterised by significantly elevat ed OPG, ACP5, RANKL, IL-6, IL-1β, DKK-1, and an increased RANKL/OPG ratio, remaining significant after false discovery rate correction. Subgroup analyses across osteoporosis, osteopenia, and normal bone status showed consistent elevation of DKK-1 in SSc patients, with additional subgroup-specific differences of OPG, ACP5, RANKL, IL-6, and IL-1β levels, which were preferentially featured with osteoporosis and osteopenia. Discussion Early diagnosis and molecular characterisation of bone metabolism alterations may be crucial for optimising therapeutic strategies and potentially reducing the extent of bone loss in patients with SSc. Conclusions In women with relatively early SSc, a distinct osteoimmunological biomarker profile differentiated SSc from controls and was associated with DXA-derived BMD parameters, supporting a link between osteoimmunological alterations and skeletal involvement in the first years of SSc.

Introduction Systemic sclerosis (SSc) is characterised by microvascular abnormalities affecting multiple organs. Pain is a prevalent and debilitating symptom of SSc that can have a significant impact on patients’ quality of life and physical function. The study aims to evaluate functional impairment in patients with SSc and to investigate their association with disease peculiarities and pain. Material and Methods A retrospective analysis of medical records from the European Alliance of Associations for Rheumatology (EULAR) Research Voucher grant “The bone and muscle state in patients with systemic sclerosis” included 32 patients with SSc (mean age: 57.1 ±12.5 years; body mass index: 24.6 ±4.6 kg/m²; disease duration 8.4 ±5.9 years). Functional status was assessed using HAQDI, and patients were stratified into 3 groups: G1 minimal (score 0–1), G2 moderate (1–2), and G3 severe (2–3) disability. We analysed pain in patients using the Visual Analogue Scale (VAS) and the VAS physician global score. A hand-grip dynamometer assessed muscle strength. The analysis included laboratory markers (C-reactive protein, erythrocyte sedimentation rate, interleukin-6), skin involvement evaluated by modified Rodnan skin score (mRSS), the presence of digital ulcers (DU) and interstitial lung disease (ILD). Statistical analysis was performed on the Med-Stat software. Results Patients in G1 exhibited significantly lower pain scores than those in G2, as measured by both the patient VAS (30.1 ±18.1 vs. 59.0 ±18.5; p = 0.05) and the physician VAS (26.9 ±16.0 vs. 50.5 ±19.2; p = 0.05). As far as it was in G3: VAS patient (63.3 ±15.1 vs. 30.1 ±18.1; p = 0.05), and VAS physician (69.3 ±34.4 vs. 26.9 ±16.0; p = 0.05). Among G3 patients in comparison with G1, significantly higher mRSS scores (27.3 ±7.9 vs. 16.7 ±7.8; p = 0.05) and reduced hand grip strength (6.33 ±6.1 vs. 16.7 ±7.8; p = 0.05) were demonstrated. No significant associations were observed between HAQ-DI and age, disease duration, laboratory parameters, DU or ILD. Discussion These findings highlight the importance of a thorough evaluation of functional limitations and pain in SSc. Our observations emphasise that functional impairments are closely related to patients’ reported pain and the severity of the disease as assessed by physicians. In our study, decreased hand strength and skin involvement were found to be risk factors for functional impairment. Conclusions In patients with SSc, functional impairment correlates with pain intensity, muscle strength, extent of skin involvement, and global disease activity assessed by physicians

Introduction Systemic sclerosis (SSc) is a chronic autoimmune disease characterised by immune dysregulation, vasculopathy, and progressive fibrosis. Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a leading cause of morbidity and mortality. Early identification of clinical and microvascular markers linked to ILD is crucial for risk stratification and timely intervention. The study aims to evaluate risk factors associated with the presence of SSc-ILD through comparative analysis of clinical, functional, serological, echocardiographic and microvascular parameters. Material and Methods This study analysed 41 adult patients with SSc (American College of Rheumatology/European Alliance of Associations for Rheumatology 2013) treated at the EUSTAR centre in Kyiv (2022–2024). Patients were stratified into ILD and non-ILD groups based on high-resolution computed tomography. Variables assessed: SSc subtype, modified Rodnan Skin Score (mRSS), autoantibodies (anti-Scl-70, ACA), pulmonary function (forced vital capacity [FVC], diffusion capacity of the lungs for carbon monoxide), 6-minute walk test (6MWT), and echocardiographic indices (left ventricular ejection fraction, E/A ratio). Microvascular damage was evaluated via nailfold videocapillaroscopy (NVC) using Cutolo patterns. Statistical analysis employed Mann-Whitney U and Fisher’s exact tests (p = 0.05). Results The cohort was predominantly female (mean age 56.2 ± 12.4 years; disease duration 8.9 ±6.4 years). Interstitial lung disease was present in 63.4% of patients and was significantly associated with diffuse cutaneous SSc (96.2% vs. 26.7%; p = 0.001), higher mRSS (22.9 ±8.9 vs. 15.5 ±7.4; p = 0.007), and anti-Scl-70 positivity (57.7% vs. 19.2%; p = 0.024). The ILD patients showed lower FVC (74.9% vs. 96.5%; p = 0.010) and shorter 6MWT distance (281.5 ±64.5 m vs. 396.7 ±59.5 m; p = 0.001). An E/A ratio 0.8 was more frequent in ILD (57.7% vs. 20.0%; p = 0.025). Interstitial lung disease was also associated with advanced NVC patterns, reduced capillary density, and more frequent digital ulcers (69.2% vs. 33.3%; p = 0.049). Discussion The SSc-ILD is associated with a severe systemic phenotype marked by skin fibrosis and anti-Scl-70 positivity. Reduced FVC and 6MWT indicate functional impairment, while the higher prevalence of E/A 0.8 suggests early diastolic dysfunction may contribute to exercise intolerance. The association between advanced NVC patterns and ILD supports the link between microvascular damage and internal organ involvement. Conclusions In SSc, ILD identifies a high-risk subgroup with functional decline and advanced vasculopathy. Integrated assessment, including pulmonary, cardiac, and microvascular evaluation, is crucial for early risk stratification and personalised management.

Systemic sclerosis is a complex autoimmune disease characterized by vasculopathy and fibrosis, capable of affecting virtually any organ system, including the eyes. Optical coherence tomography is a non - invasive imaging technique that enables high-resolution cross-sectional visualization of ocular structures. This study aimed to characterize candidate retinal imaging features in systemic sclerosis patients using Optical Coherence Tomography. A total of 41 systemic sclerosis patients (10 males, 31 females; mean age: 58.7 ± 11.7 years) and 38 age-matched healthy controls (9 males, 29 females; 61.6 ± 12.8 years) were included. After all participants underwent a comprehensive ophthalmologic assessment, including best-corrected visual acuity, intraocular pressure measurement, slit-lamp biomicroscopy, fundus examination, and optical coherence tomography was applied. The mean best-corrected visual acuity among systemic sclerosis patients was 0.95. The central retinal thickness was slightly reduced in systemic sclerosis patients (241.7 µm) compared to controls (254.9 µm), though the difference was not statistically significant. Subfoveal choroidal thickness showed no significant difference (289.9 µm vs. 285.4 µm in controls). Notably, optical coherence tomography revealed previously unreported fundus abnormalities in systemic sclerosis patients: drusen-like deposits in 19 patients (46.3%), wide-based foveal pit in 26 patients (63%), and epiretinal membrane in 9 patients (21%), all of which occurred significantly more often than in the control group (p < 0.01). Systemic sclerosis is associated with a high prevalence of underrecognized retinal findings, like foveal contour abnormalities and drusen-like deposits. While these candidate imaging features require longitudinal validation, they likely reflect the systemic microvascular and fibrotic burden of the disease.