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  • Ischemic Digital Ulcers
  • Ischemic Digital Ulcers
  • Digital Pitting
  • Digital Pitting

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  • Research Article
  • 10.1007/s00296-026-06207-z
Combined mechanical and chemical sympathectomy for refractory complicated Raynaud's phenomenon: an international multicenter case-based review.
  • Jun 22, 2026
  • Rheumatology international
  • Oscar A De La Torre + 5 more

Secondary Raynaud's Phenomenon (RP) associated with connective tissue diseases (CTD) frequently presents as critical digital ischemia refractory to standard protocols. This review describes the clinical feasibility of a combined approach involving mechanical (surgical adventitial stripping) and chemical (Botulinum Toxin Type A [BTX-A] and/or prostanoid analogues) sympathectomy. We retrospectively reviewed four patients from tertiary centers in Mexico and Argentina presenting with severe refractory RP. Interventions involved periarterial digital sympathectomy augmented by intradigital BTX-A and/or intravenous (IV) prostanoids. Longitudinal clinical observations were recorded regarding pain reduction using the Visual Analog Scale (VAS), digital ulcer healing, and vascular imaging. Clinical remission was achieved in 3/4 patients. Case one healed a 0.5cm necrotic lesion following bilateral stripping and 80IU BTX-A. Case two, achieved complete tissue stabilization at 16-month follow-up; intraoperative angiography confirmed the immediate restoration of digital arch perfusion post-intervention. Case three, presented with a "late" capillaroscopy pattern, maintained a 60-month ulcer-free interval using 80IU BTX-A and Alprostadil. Case four involved 8/10 ischemic digits; the course was complicated by a radial artery thrombosis requiring a radio-radial venous graft bypass. This patient suffered a relapse with three new distal ulcers immediately following an administrative gap in Bosentan therapy. These hypothesis-generating findings suggest that multimodal sympathectomy is a feasible adjunctive strategy for limb salvage in refractory complicated secondary RP. Current literature enforces these findings supporting the notion for standardized dosing and stepwise approach for future research to support these hypothesis generating views.

  • Research Article
  • 10.5606/tftrd.2026.16356
Evaluation of hand functional indices in association to clinical, laboratory, and radiologic data in patients with systemic sclerosis
  • Jun 10, 2026
  • Turkish Journal of Physical Medicine and Rehabilitation
  • Ayşegül Yetişir + 4 more

This study aims to compare the clinical, radiological, and general health status of patients with systemic sclerosis (SSc) using the Duruöz Hand Index (DHI) and the modified Hand Mobility in Scleroderma (mHAMIS) test. This cross-sectional, single-center study included 71 patients (68 females, 3 males; mean age: 49.1± 8.1 years; range, 31 to 69 years) with SSc between January 2024 and August 2024. We recorded sociodemographic data, disease-related parameters, laboratory parameters, medications, physical examination findings of the hand, direct radiographic changes, and the degree of skin stiffness with the modified Rodnan skin score (mRSS) in patients with SSc. Hand function was assessed with two hand indices: DHI and mHAMIS. Patients' general health status was evaluated with the Health Assessment Questionnaire-Disability Index (HAQ-DI). The mean DHI and the median total mHAMIS scores were 16.7 ± 15.8 and 1 (4), respectively. Patients showed a moderate to strong positive correlation of both DHI and mHAMIS scores with since time to diagnosis, diffuse cutaneous SSc, mRSS, hand examination, radiography, and the HAQ-DI scores, and a negative correlation with body mass index (p<0.05). Digital ulcers and erosive changes were associated with DHI scores, while the since time to diagnosis, digital ulcers, and flexion contracture on radiographic examination affected mHAMIS scores in multiple regression analysis. The mHAMIS score showed a higher coefficient correlation value with disease-related parameters, hand examination, radiography, mRSS, and HAQ-DI scores compared to the DHI score. The findings demonstrated that mHAMIS, a simple tool for assessing patient hand functionality, more accurately captured hand examination and radiography, disease-related parameters, mRSS, and HAQ-DI scores.

  • Research Article
  • 10.1136/rmdopen-2025-006688
Safety and effectiveness of tocilizumab in systemic sclerosis: a multicentre French-Italian study.
  • Jun 9, 2026
  • RMD open
  • Giulia Buonsante + 29 more

Tocilizumab (TCZ) has shown beneficial effects on interstitial lung disease (ILD) in systemic sclerosis (SSc). We aimed to assess the real-life safety and effectiveness of TCZ on several SSc-related domains using data from a French-Italian multicentre cohort. We conducted a retrospective analysis of patients with SSc treated with TCZ across 15 referral centres. The following clinical data were collected at 12 months before TCZ initiation, at baseline and at 12 and 24 months of treatment: modified Rodnan skin score (mRSS), pulmonary function tests, Disease Activity Score in 28 joints using C reactive protein, digital ulcers and cardiac biomarkers. ILD progression was defined as a decline ≥5 in %predicted forced vital capacity (%pFVC) over 12±3 months. 197 patients were included (88% female; median age 57 years; median disease duration 9 years); 67% were antitopoisomerase I positive. TCZ monotherapy was used in 29% of cases, methotrexate was the most frequent combined treatment (35%). In SSc-associated ILD, %pFVC declined significantly from -12 months to baseline (81% to 77%; p=0.003). On TCZ introduction, %pFVC stabilised and the proportion of progressors declined from 43% to 24% (p=0.015). Among diffuse cutaneous patients, mRSS decreased significantly at 12 and 24 months. Digital ulcers, arthritis activity and cardiac biomarkers also improved. Infections were the most frequent adverse events (22.8%). TCZ was discontinued in 32% of patients, mainly for inefficacy. Pulmonary arterial hypertension and older age predicted TCZ failure, whereas elevated CRP predicted better response. In this large real-life cohort, TCZ was safe and associated with consistent benefits across different domains, supporting its role as a potential disease-modifying treatment in selected patients with SSc.

  • Research Article
  • 10.1016/j.semarthrit.2026.153009
Identifying candidate core domains for clinical trials in systemic sclerosis-associated Raynaud's phenomenon and digital ulcers.
  • Jun 4, 2026
  • Seminars in arthritis and rheumatism
  • Susanna M Proudman + 11 more

Identifying candidate core domains for clinical trials in systemic sclerosis-associated Raynaud's phenomenon and digital ulcers.

  • Research Article
  • 10.1093/rheumatology/keag267
The clinical utility of telangiectasia quantification as markers of vascular disease progression in systemic sclerosis.
  • Jun 3, 2026
  • Rheumatology (Oxford, England)
  • Matthew Wells + 11 more

Telangiectasia are common in SSc. We explored the relationship between the site and quantity of telangiectasia with disease characteristics in SSc, and the agreement between patient- and physician-reported quantification of telangiectasia. A retrospective analysis of a large, cross-sectional international SSc-related vasculopathy study was undertaken, including clinician and patient assessments of telangiectasia counts (0, 1-6, 7-15 or >15) in the face, forearms and hands. Relationships between telangiectasia count at each site, demographics and clinical features including calcinosis, digital ulceration (DU) and pulmonary arterial hypertension (PAH) were examined using proportional odds logistic regression. A binary logistic regression model examined the value of telangiectasia counts on the accuracy of identifying co-existent PAH. Concordance between clinician- and patient-reported telangiectasia counts at each anatomical site was tested. Higher telangiectasia counts over the face and hands were associated with higher prevalence of calcinosis, DU and PAH in univariate analysis (P < 0.001-0.003). In multivariate analysis, the presence of PAH, DU and calcinosis were associated with higher facial telangiectasia (P < 0.05). The logistic regression model for the detection of PAH was enhanced with inclusion of telangiectasia count and site (area under the precision recall curve 0.824-0.875). Strength of agreement between clinician- and patient-reported telangiectasia counts were moderate for face and forearms (kappa 0.648 and 0.605 respectively, P < 0.001) and relatively weaker for hands (kappa 0.584, P < 0.001). The number of telangiectasia might be considered a complementary biomarker to the presence of vascular complications of SSc including PAH, DU and calcinosis. The anatomical regions and level of instruction provided for patient-reported count of telangiectasia need further optimization to be considered reliable and feasible. In addition, future work should consider correlations with nailfold capillaroscopic patterns.

  • Research Article
  • 10.1002/mus.70304
A Rare Association of Anti-SRP Antibody-Positive Immune-Mediated Necrotizing Myopathy and Sjögren Disease: Two Case Reports.
  • Jun 1, 2026
  • Muscle & nerve
  • Hiroaki Tanaka + 6 more

Immune-mediated necrotizing myopathy (IMNM) is an autoimmune myositis which is characterized by severe muscle fiber necrosis and refractoriness to immunotherapies. Overlap between myositis and other autoimmune disorders is well-recognized; however, the coexistence of IMNM and Sjögren disease (SjD) is rare. We investigated the clinicopathological features of anti-signal recognition particle antibody-positive IMNM associated with SjD. We retrospectively reviewed previously reported cases and analyzed the clinical and pathological features of two patients: a 68-year-old man with a 2-year history of sicca symptoms, who presented a 3-month history of dysphagia, difficulty arising from a sitting position and gait disturbance, and a 67-year-old woman with a 20-year history of sicca symptoms, who subsequently developed muscle weakness, dysphagia, and hyperCKemia. Muscle pathological findings in both patients were consistent with those of IMNM without SjD. In contrast, they exhibited relatively mild limb weakness and showed a better response to immunotherapies compared with patients with typical IMNM. Notably, Patient 1 developed severe Raynaud's phenomenon and digital ulcers, requiring additional treatment. IMNM associated with SjD may present with relatively mild muscle involvement compared with IMNM without SjD; however, SjD-related disease activity may become more prominent during the clinical course.

  • Research Article
  • 10.1016/j.semarthrit.2026.152979
Systemic sclerosis-related digital ulcers: Current understanding and updated management approaches- a primer for clinicians.
  • Jun 1, 2026
  • Seminars in arthritis and rheumatism
  • Gulsen Ozen + 2 more

Systemic sclerosis-related digital ulcers: Current understanding and updated management approaches- a primer for clinicians.

  • Research Article
  • 10.1016/j.jaut.2026.103574
Mechanistic studies on the regulation of systemic sclerosis progression by imbalance in angiogenic T cell subsets.
  • Jun 1, 2026
  • Journal of autoimmunity
  • Haomiao Shen + 4 more

Mechanistic studies on the regulation of systemic sclerosis progression by imbalance in angiogenic T cell subsets.

  • Research Article
  • 10.1007/s10067-026-08168-x
Longitudinal clinical response to Janus kinase inhibitors in systemic sclerosis: a real-life multicentric study across multiple clinical domains.
  • May 30, 2026
  • Clinical rheumatology
  • Stefano Di Donato + 8 more

Janus kinase inhibitors (JAKi's) have shown promising results in systemic sclerosis (SSc), yet little studied. We evaluated safety and intra-patient changes in pulmonary, articular, and cutaneous parameters in SSc JAKi-treated patients. An international, multi-centre, longitudinal retrospective cohort study. We assessed changes in FVC%, DLCO%, modified Rodnan skin score (mRSS), tender/swollen joint counts (TJC/SJC), digital ulcers (DU), and calcinosis. Baseline was defined as JAKi initiation. Outcomes were analysed as delta from baseline using Wilcoxon signed-rank tests, with effect sizes expressed as standardized mean change (SMC). Among 32 patients treated with the four available JAKi's, median (IQR) follow-up was 16.9 (10.3, 31.8) months, totalling 52.8 patient-years. At 12months, pulmonary function remained stable (SMC = + 0.22 for FVC%, + 0.23 for DLCO%; p = 0.10 and p = 0.33, respectively). mRSS (SMC = - 0.29, p = 0.03), TJC and SJC (SMC = - 1.19 and - 0.69, p < 0.001 and p = 0.001, respectively) significantly improved. At 24months, numerical improvements in mRSS, TJC, and SJC persisted with SMC of - 0.21, - 1.13, and - 1.14 (p = 0.17, p = 0.054, p = 0.058, respectively). Among 11 patients with baseline calcinosis, 45.5% improved. Ten patients developed DUs. In 15 patients receiving glucocorticoids, a non-significant trend toward tapering was observed. JAKi treatment in SSc patients was associated with 'real-world' improvements in multiple domains; findings are exploratory. Key Points • JAKi treatment in SSc patients was associated with 'real-world' improvements in multiple clinically important domains. • Relevant safety concerns with JAKi treatment were confirmed in our treated SSc patient cohort. • Our findings provide preliminary real-world evidence supporting a potential role of JAK inhibitors across multiple clinical domains in SSc. • Long-term controlled trials to confirm the safety and efficacy of JAKi's in patients with SSc are needed.

  • Research Article
  • 10.1136/jsrd-2026-000005
Knowledge and perceptions of medical practitioners on the role of nutrition in systemic sclerosis
  • May 26, 2026
  • Journal of Scleroderma and Related Disorders
  • Elisavet Efthymiou + 9 more

ObjectivesSystemic sclerosis (SSc) presents complex manifestations that impact disease progression and quality of life. Despite the burden of gastrointestinal (GI) and nutrition-related symptoms, a lack of consensus is apparent regarding the appropriate dietary strategies to support SSc management. The present study aimed to explore the perspectives of rheumatologists regarding the importance of nutrition counselling and nutrition-related patients’ needs.MethodsA cross-sectional study was conducted using a sample of 74 rheumatologists in Greece. The Views on Education and Nutrition in Scleroderma (VENUS) questionnaire was employed. The tool assesses three domains: demographics, scleroderma management and nutrition education approaches. Descriptive statistics summarised the findings, while comparisons and correlations were performed, where appropriate.ResultsPhysicians noted that patients with SSc requested dietary advice for symptom control (58.1%), improving general nutrition knowledge (32.4%) and adhering to specialised diets (29.7%). While practitioners recognised the importance of nutrition in SSc care, they emphasised the value of structured support, including face-to-face consultations (51.4%) and the integration of dietitians into multidisciplinary care teams (40.5%). Patients frequently sought nutritional advice, especially regarding reflux management (74.3%), soft-food diet (47.0%) and less commonly other dietary regimens. Regarding symptomatology, most rheumatologists reported pain, digital ulcers and GI discomfort in over half of their patients. Most commonly reported GI symptoms included reflux (89.2%), bloating (55.4%), heartburn (54.1%) and dysphagia (52.7%).ConclusionPatients with SSc experience significant pain and GI disturbances and actively seek dietary guidance. Rheumatologists acknowledge the importance of nutrition in disease management and highlight the need for comprehensive multidisciplinary care models involving dietitians, aiming to improve patient outcomes.

  • Research Article
  • 10.1136/rmdopen-2025-006495
Clinical and serological features of triple autoantibody-negative patients with systemic sclerosis: insights from the multicentric SPRING registry of the Italian Society for Rheumatology
  • May 13, 2026
  • RMD Open
  • Veronica Batani + 76 more

BackgroundAntitopoisomerase I (ATA), anticentromere (ACA) and anti-RNA polymerase III (RNAP3) antibodies are included in the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for systemic sclerosis (SSc). A subset of patients with SSc satisfy criteria but may lack these specific autoantibodies, being classified as ‘triple-negative’.MethodsWe conducted a retrospective evaluation of triple-negative patients with SSc prevalence and clinical features among the multicentric Systemic sclerosis Progression INvestiGation registry.ResultsOut of 1480 patients with SSc, 295 (19.9%) were triple-negative, while 1185 (81.1%) had SSc-specific antibodies: ACA (54.3%), ATA (43.6%) and RNAP3 (2.1%). The triple-negative group showed a higher prevalence of myopathy (16.7% vs 10.1%, p=0.003), suggested by higher creatine phosphokinase (CPK) levels (126.2 vs 92.5 U/mL, p=0.002), more frequent CPK increase over 2–3 times (2.4% vs 0.2%, p=0.028). Triple-negative patients also exhibited fewer vascular complications, including digital ulcers (17.3% vs 22.8%, p=0.04) and calcinosis (8.2% vs 12.8%, p=0.027), and a higher prevalence of interstitial lung disease (p<0.001). Consistently, lower diffusing capacity for carbon monoxide (66.4% vs 70.98%, p=0.004) and forced vital capacity (97.01% vs 102.92%, p<0.001) were found in the triple-negative group. Triple-negative patients more frequently received corticosteroids (79.3% vs 67.9%, p=0.003), cyclophosphamide (43.4% vs 26%, p<0.001) and azathioprine (38.5% vs 22.3%, p=0.002), while less frequently received prostanoids (71.6% vs 85.9%, p<0.001), calcium channel blockers (80.1% vs 87.7%, p=0.005) and phosphodiesterase-5 inhibitors (4% vs 20%, p<0.001).ConclusionsA higher prevalence of myopathy and interstitial lung disease and a reduced vascular burden were found in the triple-negative patients, suggesting that the non-specific and non-routinely tested autoantibodies may identify an SSc endotype resembling sclero-myositis.

  • Research Article
  • 10.1093/rheumatology/keag176
Clinical characteristics and risk factors for calcinosis cutis in systemic sclerosis: insights from the EUSTAR database.
  • May 5, 2026
  • Rheumatology (Oxford, England)
  • Aslihan Avanoglu-Guler + 20 more

Calcinosis Cutis (CaC) represents one of the most frequent and disabling non-lethal manifestations in SSc. Our objectives were to evaluate (1) associations of CaC with SSc clinical characteristics and (2) identify risk factors for CaC development. EUSTAR database-registered SSc patients with available information on their CaC status were included. We compared baseline patient characteristics (with vs without CaC at baseline) and investigated predictors of CaC development at 5 and 10 years (in those without baseline CaC) with logistic regression analyses. A total of 7114 SSc patients were included. At baseline, 11.9% had CaC. Among 1010 and 997 patients without baseline CaC, 40% and 46% developed CaC within 5 years and 10 years, respectively. Patients with CaC were more frequently female, had longer disease duration, higher modified Rodnan Skin Score, telangiectasia, digital ischaemia, late capillaroscopy patterns, joint contractures, tendon friction rubs, gastrointestinal involvement (all P < 0.001), pulmonary arterial hypertension (PAH) (P = 0.02), joint synovitis, and renal crisis (both P = 0.04). CaC patients had a higher frequency of ACA and anti-PM/Scl antibody positivity (P < 0.001; P = 0.03, respectively). Predictors for the development of CaC at 5 years included longer disease duration [odds ratio (OR) 1.04], cardiac involvement (OR 1.63), late capillaroscopy pattern (OR 1.70), telangiectasia (OR 1.92), digital ulcers (OR 2.60), and PAH (OR 2.10). Predictors at 10 years included longer disease duration (OR 1.03), dcSSc (OR 1.51), female gender (OR 1.85), telangiectasia (OR 1.92), and digital ulcers (OR 2.92). CaC is common and progressive; clinical risk factors may provide insights into the pathogenesis.

  • Research Article
  • 10.1016/j.autrev.2026.104041
Prevalence and clinical significance of anti-NOR90 antibodies in systemic sclerosis: Results from a multicentre cohort and systematic literature review.
  • May 1, 2026
  • Autoimmunity reviews
  • Javier Narváez + 11 more

Prevalence and clinical significance of anti-NOR90 antibodies in systemic sclerosis: Results from a multicentre cohort and systematic literature review.

  • Research Article
  • 10.1016/j.berh.2026.102122
Physical rehabilitation interventions for hand function in people with systemic sclerosis.
  • May 1, 2026
  • Best practice & research. Clinical rheumatology
  • Michael Denton + 2 more

Physical rehabilitation interventions for hand function in people with systemic sclerosis.

  • Research Article
  • 10.1016/j.berh.2026.102130
Nailfold capillaroscopy and organ involvement in systemic sclerosis: a systematic review.
  • May 1, 2026
  • Best practice & research. Clinical rheumatology
  • C Ickinger + 7 more

Nailfold capillaroscopy and organ involvement in systemic sclerosis: a systematic review.

  • Research Article
  • 10.1172/jci.insight.189107
FOSL2 regulates endothelial cell state and chromatin accessibility in systemic sclerosis pulmonary vascular remodeling.
  • Apr 22, 2026
  • JCI insight
  • Rithika Behera + 18 more

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.

  • Research Article
  • 10.5114/reum/219195
Functional impairment in patients with systemic sclerosis: relation to disease peculiarities and pain
  • Apr 21, 2026
  • Rheumatology
  • Yaroslava Krasienko + 1 more

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

  • Research Article
  • 10.5114/reum/219180
Clinical, functional, and microvascular predictors of interstitial lung disease in systemic sclerosis
  • Apr 21, 2026
  • Rheumatology
  • Marta Dzhus + 5 more

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.

  • Research Article
  • 10.55563/clinexprheumatol/pfen40
Evaluation of particle-based multi-analyte technology for autoantibody detection in systemic sclerosis: concordance with conventional methods and clinical associations.
  • Apr 20, 2026
  • Clinical and experimental rheumatology
  • Albert Pérez-Isidro + 10 more

To evaluate the clinical performance of particle-based multi-analyte technology (PMAT) for the detection of systemic sclerosis (SSc)-associated autoantibodies and to explore correlations between antibody profiles and disease manifestations. We assessed PMAT performance in 124 SSc patients fulfilling the 2013 ACR/EULAR criteria, 205 disease controls, and 25 healthy individuals. All samples were tested for a broad autoantibody panel, including both classification and non-criteria autoantibodies. Concordance with conventional methods (chemiluminescence and ELISA) and associations with clinical features were analysed. PMAT showed excellent agreement with established techniques for anti-centromere autoantibodies (ACA) (κ=0.95) and anti-topoisomerase I (anti-Topo I) (κ=0.95), and good agreement for anti-RNA polymerase III autoantibodies (anti-RNApol III) (κ=0.78), confirming its reliability in detecting major SSc autoantibodies. The most prevalent autoantibodies were ACA (54.0%), anti-Topo I (21.0%), and anti-RNApol III (5.7%), each associated with distinct clinical phenotypes: ACA with limited cutaneous SSc and absence of interstitial lung disease (ILD); anti-Topo I with diffuse cutaneous SSc, ILD, and scleroderma renal crisis; and anti-RNApol III with digital ulcers and calcinosis. Importantly, novel associations were identified between PUF60 isoform 6 and severe ILD, and between PUF60 isoform 1 and the sine scleroderma phenotype, suggesting their potential as emerging biomarkers. These findings support the diagnostic and clinical utility of comprehensive serological profiling and the integration of PMAT into routine diagnostic workflows. The identification of novel autoantibody-phenotype associations underscore the value of multiplex technologies in advancing precision medicine for SSc.

  • Research Article
  • 10.1097/prs.0000000000013126
Vasospastic and Ischemic Disorders of the Upper Extremity: Vascular Radiology Collaborations Guiding Raynaud's Treatment Strategies.
  • Apr 16, 2026
  • Plastic and reconstructive surgery
  • Elliot L H Le + 7 more

Raynaud's phenomenon (RP) encompasses vasospastic disorders that can progress to digital ischemia, ulceration, and tissue loss. Despite multiple therapeutic options, variability in diagnostic evaluation and procedural indications can delay appropriate treatment. This review summarizes current evidence on RP and presents a structured diagnostic pathway and treatment algorithm for patients with vasospastic disease and digital ischemia. A review of contemporary literature on the epidemiology, pathophysiology, diagnostic studies and therapeutic interventions for RP was collected. Additionally, we describe our institution's algorithm which integrates noninvasive vascular diagnostics, catheter angiography with vasodilation challenges and a stepwise approach to procedure selection based on patterns of vasospasm and occlusion. Primary RP is a vasospastic disorder whereas secondary RP involves vasospasm and fixed occlusive disease. Noninvasive studies help assess wound healing potential and abnormal results will lead to an interventional radiology consultation for catheter angiography. Angiography defines the level of disease, responsiveness to vasodilators and suitability for intervention. Interventions include chemical and mechanical sympathectomy which is most suitable in patients with vasospastic disease and less effective in secondary RP. Patients with segmental occlusions and patent distal targets or with secondary RP will benefit from open arterial bypass or arterial reconstruction. Patients with disease isolated to the proper digital arteries are not amenable to targeted surgical interventions due to the risk of worsening ischemia. A structured, physiology-guided diagnostic and treatment algorithm can improve the precision of RP management. Integrating noninvasive testing with angiography allows tailored use of sympathectomy and reconstruction in these patients.

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