OBJECTIVE:The treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD) has evolved with increasingly available immunosuppressive therapies (ISTs) and antifibrotic treatments. However, their real-world use remains unclear. The objective of this study was to analyze treatment trends and the effect of IST and antifibrotic treatments on ILD progression using the European Scleroderma Trials and Research database. METHODS:We included patients with SSc-ILD meeting the 2013 American College of Rheumatology/EULAR criteria with high-resolution computed tomography-confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007-2011, 2012-2016, and ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in the percentage of predicted forced vital capacity of 5% or greater or the percentage of predicted diffusing capacity of the lungs for carbon monoxide of 10% or greater over 12 ± 3 months. RESULTS:Among 1,409 patients, IST use at first evaluation increased significantly from 13.6% (≤2006) to 57.4% (≥2017) (P < 0.001). Mycophenolate mofetil emerged as the most prescribed IST (7% to 57%) (P < 0.001). Combination therapy rose from 17.9% to 26.9% (P < 0.001), whereas ILD progression rates declined from 21.3% (2007-2011) to 12.1% (≥2017) (P < 0.001). In the 2017 and later cohort, logistic regression showed shorter disease duration (odds ratio [OR] 0.991, 95% confidence interval [CI] 0.987-0.996; P < 0.001) and myositis (OR 9.9, 95% CI 1.94-51.76; P = 0.006) were associated with therapy initiation, whereas switching was higher in patients with a higher modified Rodnan skin score (OR 1.03, 95% CI 1.00-1.06; P = 0.035) and in patients with arthritis (OR 3.03, 95% CI 1.55-5.94; P = 0.001). Last, combination therapy was associated with younger age, higher dyspnea class, and arthritis. CONCLUSION:Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches.

INTRODUCTION:Assessment of disease activity in juvenile systemic sclerosis (jSSc) is essential for clinical care and trial readiness, yet no validated pediatric activity measures exist. Adult systemic sclerosis activity tools, including the Scleroderma Clinical Trials Consortium Activity Index, revised CRISS, and revised EUSTAR, provide conceptual frameworks but require adaptation for developmental, physiologic, and feasibility considerations for children. At the 17th Hamburg Symposium on JSSc, an international multidisciplinary panel reviewed adult indices, evaluated corresponding variables in the jSSc Inception Cohort and the NRCOS registry, and conducted a structured Delphi process to define organ-specific indicators of clinically meaningful activity in jSSc. AREAS COVERED:Across skin, pulmonary, cardiac, vascular, musculoskeletal, gastrointestinal, renal, and global domains, the panel reached broad and often unanimous consensus on variables reflecting active, potentially reversible disease. Key endorsed measures included mRSS progression, new ILD on HRCT, ≥10% declines in FVC or DLCO, new cardiac abnormalities, active digital ulcers, synovitis, myositis, nutritional decline, and physician global assessment. Patient-reported outcomes were strongly supported across domains. EXPERT OPINION:These consensus-derived indicators provide the first comprehensive pediatric-specific foundation for defining disease activity in jSSc and represent a critical step toward developing and validating a unified pediatric activity index suitable for future clinical trials.

Background Interstitial lung disease (ILD) is a major pulmonary complication of idiopathic inflammatory myopathy (IIM), where early diagnosis improves outcomes. While high-resolution CT (HRCT) remains the gold standard, its radiation exposure poses concerns. Serum Krebs von den Lungen-6 (KL-6) and lung ultrasound (LUS) B-lines offer non-invasive alternatives, though their optimal diagnostic cut-offs and combined utility for IIM-ILD remain undefined. This study aimed to establish these cut-offs, evaluate diagnostic performance and develop a clinical prediction model.Methods In this single-centre observational study, 162 patients diagnosed with IIM between 2020 and 2024 were enrolled. All underwent serum KL-6 testing, and 120 received systematic 50-point LUS examinations. Using HRCT as the reference standard for ILD diagnosis, receiver operating characteristics (ROC) curve analysis was used to determine optimal cut-offs and construct a combined nomogram.Results KL-6 levels were significantly elevated in the ILD group (n=113) compared with non-ILD (n=49). The optimal KL-6 cut-off was 553 U/mL (area under the curve (AUC)=0.895, sensitivity 69%, specificity 95.9%). For LUS B-lines number, 25 was recommended as the clinical threshold (sensitivity 98.8%). Their combination enhanced diagnostic performance (AUC=0.984). An online prediction model demonstrated strong clinical applicability. In an exploratory analysis of the rapid-progressive ILD subgroups, KL-6 and B-lines showed only modest predictive value (AUC ≈ 0.65).Conclusion KL-6 ≥553 U/mL and B-lines ≥25 are effective screening thresholds, with combined use significantly improving diagnostic accuracy. The prediction model provides a practical tool for early identification in similar clinical settings. To optimise and generalise its use, external validation in multicentre cohorts is warranted.

Objectives To assess the construct validity of a modified single-item measure of bother due to side effects (the GP5 item) from the Functional Assessment of Chronic Illness Therapy (FACIT) system by comparing it to current symptomatic side effects from the Patient-Reported Outcomes of the Common Terminology Criteria for Adverse Events (PRO-CTCAE) reported by patients with rheumatoid arthritis (RA). Methods Through a cross-sectional, web-based survey we collected information on the frequency of symptomatic side effects and bother from side effects related to RA medications. We applied multiple correspondence analysis (MCA) to reduce 80 symptomatic side effects into key dimensions (≥5% of the total variance each). We then examined associations among key dimensions, individual items, the sum of current side effects, and the single-item bother measure using Spearman rho. Results A total of 560 patients participated in the online survey. Our scree plot showed a clear elbow point after the first dimension, indicating that keeping just one dimension captured the most meaningful information. This overall side effect burden score appeared to reflect a broad concept influenced by a variety of symptomatic side effects, each having only a negligible to weak impact. Conclusions Our results may indicate that individuals have diverse experiences of side effects, allowing the global index to capture these variations, even when they differ across patients. Thus, a single-item burden measure to side effects can potentially serve as a useful summary indicator, shedding light on the impact of symptomatic side effects experienced by RA patients.

OBJECTIVES:To build on existing evidence regarding single-item measurement instruments of patient-reported bother or trouble from medical side effects in individuals with rheumatic and musculoskeletal diseases (RMDs). Further, to collect input from the OMERACT community through a structured survey that rated and ranked available options and to seek agreement to advance one or more of these measures for use as exploratory outcomes in future clinical trials. METHODS:At OMERACT 2025 we presented and discussed survey results for domain match, feasibility and ranking of six candidate instruments of bother or trouble from side effects. Collaborator feedback - including comments from patients, clinicians, and researchers - was synthesized with a large-language-model (LLM) to identify key concerns and guide refinement of the instrument's relevance, clarity, and acceptability. The LLM-assisted synthesis of participant comments resulted in a new, single-item instrument designed to improve patient safety reporting from the patient's perspective. RESULTS:The merged and modified version of the instrument was presented at the OMERACT 2025 meeting, where 33 participants approved it as a reasonable approach to incorporate collaborator input. The proposed instrument is feasible (32 [97%]) and voting supported advancing its further assessment (30 [91%]) as an exploratory outcome measurement instrument in coming RMD trials. CONCLUSION:We developed a novel single-item instrument. This is the first known application of LLMs in refining a patient-reported outcome instrument for clinical trials. It is designed to capture the patient perspective on symptomatic treatment-related side effects in RMDs and is supported for exploratory use in trials.

Objectives Megakaryocytes (MKs) and low-density granulocytes (LDGs) are implicated in immune dysregulation and vascular pathology in autoimmune diseases (ADs), yet their precise subsets and pathological interactions remain poorly defined. We aimed to characterize MK and LDG subpopulations and elucidate their potential intercellular communication in ADs using single-cell transcriptomic analysis. Methods Single-cell RNA sequencing (scRNA-seq) was performed on peripheral blood mononuclear cells from 10 treatment-naive AD patients (4 pSS, 3 RA, and 3 SLE) and 3 healthy controls (HCs). MKs and LDGs were re-clustered to identify transcriptional subpopulations and interrogated for intercellular communication using CellChat. A distinct megakaryocyte-like granulocyte population was validated in an independent scRNA-seq dataset. Bulk RNA-seq (n = 139) and plasma ELISA assays were employed to support the associated molecular signatures. Crucially, flow cytometry of peripheral blood from AD patients (n = 5) and HCs (n = 4) was performed to provide protein-level validation of the identified megakaryocyte-like granulocytes. Results MKs segregated into immune-active and platelet-generating subtypes, both exhibiting altered signaling in ADs. LDGs harbored a unique PF4+/PPBP+ megakaryocyte-like subpopulation with heightened interferon activity, proinflammatory signaling, and transcriptional signatures of increased neutrophil extracellular trap (NET) formation. Flow cytometry confirmed the presence of these granulocytes and showed a higher proportion in AD patients than in HCs. These granulocytes showed predicted communication with MKs via ITGB2-ICAM2 and APP-CD74 axes. Findings were consistently validated in an external scRNA-seq dataset and corroborated by bulk RNA-seq deconvolution and elevated plasma myeloperoxidase levels. Conclusions We identify a potentially PF4+/PPBP+ megakaryocyte-like granulocyte subset associated with immune dysregulation in ADs. While flow cytometry provides protein-level evidence for this population, further mechanistic studies are required to fully elucidate its functional role in disease pathogenesis.

The treatment landscape for systemic sclerosis‐associated interstitial lung disease (SSc‐ILD) has evolved with increasing immunosuppressive (IST) and anti‐fibrotic therapies available. However, their real‐world use remains unclear. To analyze treatment trends and the effect of IST and anti‐fibrotic therapies on ILD progression using the EUSTAR database. We included SSc‐ILD patients meeting the 2013 ACR/EULAR criteria with high‐resolution CT‐confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007–2011, 2012–2016, ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in %FVC ≥5% or %DLCO ≥10% over 12 ± 3 months. Among 1,409 patients, IST use at first evaluation increased significantly from 13.6% (≤2006) to 57.4% (≥2017, p<0.001). Mycophenolate mofetil emerged as the most prescribed IST (7% to 57%, p<0.001). Combination therapy rose from 17.9% to 26.9% (p<0.001), while ILD progression rates declined from 21.3% (2007–2011) to 12.1% (≥2017, p<0.001). In the ≥2017 cohort, logistic regression showed shorter disease duration (Odds ratio (OR) 0.991, 95%CI 0.987–0.996, p <0.001) and myositis (OR 9.9, 95% CI 1.94‐51.76, p=0.006) were associated with therapy initiation, while switching was higher in patients with a higher mRSS (OR 1.03, 95%CI 1.00–1.06, p=0.035) and in patients with arthritis (OR 3.03, 95% CI 1.55–5.94, p=0.001). Lastly, combination therapy was associated with younger age, higher dyspnea class and arthritis. Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches. image

OBJECTIVES:Rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and primary Sjögren's syndrome (pSS) frequently share overlapping clinical features, yet exhibit substantial intradisease heterogeneity in organ involvement and severity. This study aimed to define conserved molecular subtypes across these systemic autoimmune diseases (SADs) and delineate their transcriptional and epigenetic underpinnings. METHODS:We profiled 262 treatment-naïve Chinese Han patients (RA: n = 109; SLE: n = 69; pSS: n = 84), predominantly female (83.2%), who were divided into discovery (n = 119) and validation (n = 143) cohorts. Bulk RNA sequencing defined molecular subtypes, which were correlated with clinical phenotypes. Chromatin accessibility and super-enhancer landscapes were mapped by Assay for Transposase-Accessible Chromatin with high-throughput sequencing and cleavage under targets and tagmentation, and single-cell RNA sequencing (scRNA-seq) was performed to delineate subtype-specific cellular compositions. RESULTS:Integrative analyses uncovered 2 robust and reproducible molecular subtypes, megakaryocyte-enriched and B-cell-enriched, which were consistent across all 3 SADs and both cohorts. The megakaryocyte-enriched subtype was associated with higher platelet counts, greater disease activity, and broader organ involvement. Epigenomic profiling identified distinct chromatin accessibility and regulatory architectures, with megakaryocyte-associated genes (eg, ZFP36L1 and RAD51B) linked to active superenhancers in the megakaryocyte-enriched subtype, and B-cell-associated genes (eg, MAF and PRDM1) in the B-cell-enriched subtype. scRNA-seq confirmed expansion of platelet-producing megakaryocytes and enhanced B-cell-activation signatures in their respective subtypes. CONCLUSIONS:Integrative multiomics profiling defines 2 conserved molecular subtypes across RA, SLE, and pSS with distinct cellular and regulatory programmes. This cross-disease taxonomy may inform precision stratification and the development of lineage-targeted therapies in SADs. Further multicentre validation and investigation of the epigenetic mechanisms underlying molecular subtypes are warranted.

This study investigated the diagnostic accuracy of lung ultrasound (LUS) and serum KL-6 levels for detecting interstitial lung disease (ILD) in Sjögren’s disease (SjD) patients. This retrospective study included 70 SjD patients evaluated at Shantou Central Hospital. All patients underwent chest high-resolution computed tomography (HRCT), LUS, and KL-6 measurement within one month. LUS was performed at 50 scanning sites. The presence and patterns of ILD were defined by HRCT findings. Serum KL-6 levels were measured using chemiluminescent enzyme immunoassay. Correlations between B-lines score, KL-6 level, and the HRCT Warrick score were analyzed. ROC curves with DeLong test and Spearman correlation analysis were performed to evaluate diagnostic efficiency and correlations with the Warrick HRCT fibrosis score. The concordance rate between LUS and HRCT was 82.86

Psychological stress impacts rheumatoid arthritis (RA) disease activity, and California’s response to the COVID-19 pandemic created historically significant stressors for patients. This study examined factors associated with changes in RA flares during the pandemic. In this cross-sectional COVID-19 RA study, patients with RA ICD-9/10 codes were emailed a questionnaire in July/November of 2020 containing questions on RA disease activity, Routine Assessment of Patient Index Data 3 (RAPID3), flare number and frequency, RA Flare Questionnaire (RA-FQ), Perceived Stress Scale 4 (PSS-4), stressors, and demographics. Age, anti-cyclic citrullinated antibody, and rheumatoid factor were extracted from medical records. Analyses examined associations between current flare status, number of flares, and changes in flare frequency with PSS-4 and stressors. Of 1138 respondents (22.6

OBJECTIVES:To determine the efficacy, safety and pharmacodynamics of belumosudil in patients with diffuse cutaneous systemic sclerosis (dcSSc) treated with background immunosuppressive therapies. METHODS:Eligible patients were randomised 1:1:1 to receive belumosudil 200 mg once daily (QD) or twice daily (BID), or placebo for 28 weeks (double-blind period). After unblinding, the patients who received belumosudil continued the same dose, whereas the patients who received placebo were re-randomised for one of the belumosudil doses for 24 weeks (open-label extension). RESULTS:Thirty-five and 31 patients were treated in the double-blind and open-label periods, respectively. The study was terminated prematurely, and target enrolment was not met. The primary end point, of CRISS score ≥0.60 at week 24, did not exhibit an efficacy signal in the belumosudil vs placebo groups [odds ratio: 1.06 (0.19-5.82; P = 0.9472) for the QD, and 0.39 (0.07-2.35; P = 0.3078) for the BID group]. Belumosudil was well tolerated and exhibited similar safety profiles in both double-blind and open-label periods. Tissue-based RNA sequencing analysis revealed FOXP3 upregulation and STAT3, IL23A and TGF-β downregulation in patients with CRISS score ≥0.60, which supported the mechanism of action of belumosudil. In blood and tissue samples, trends of decreased fibrosis biomarker levels were seen in the belumosudil-treated group vs placebo. CONCLUSION:Efficacy signal for belumosudil could not be detected. Signalling pathway modulation analysis supported the mechanism of action of belumosudil. A trend for decreased fibrosis-related biomarkers was observed in the belumosudil-treated group. TRIAL REGISTRATION:ClinicalTrials.gov, https://clinicaltrials.gov, NCT03919799.

Introduction:Digital ulcers (DUs) stand out as one of the most prevalent and clinically meaningful manifestations of systemic sclerosis (SSc) and are associated with significant morbidity. While systemic (pharmacological) therapy is currently established as the 'standard of care', effective local ulcer management remains crucial for all cases of DUs. This is particularly true for patients who cannot tolerate systemic treatments or in the case of refractory SSc-DUs. On this background, there is a pressing demand for the formulation of evidence-based guidelines to assist clinicians and patients in navigating the local treatment options for DUs. Methods:A steering committee of international experts was established by the World Scleorderma Foundation (WSF) Digital Ulcer (DU) ad hoc committee. Two systematic literature reviews on local non-surgical and surgical treatments for the management of SSc-DUs were performed to inform the development of local treatment recommendations for SSc-DUs. Consensus methodology was used to develop the final treatment recommendations. Results:Six overarching treatment principles and eight local treatment recommendations (five non-surgical and three surgical) were agreed upon for the management of SSc-DU. Among topical non-surgical options, botulin toxin can be conditionally recommended for refractory and/or severe DUs. Among surgical treatments, autologous adipose tissue grafting might be recommended for DU healing when combined with background systemic treatments. Conclusion:These recommendations are specifically tailored to guide treatment decisions concerning both local and non-pharmacological approaches to managing SSc-related DUs. Our work has highlighted a notable quality gap in comparison to systemic treatments, underscoring the scarcity of high-quality studies concerning this topic.

Objectives The RESOLVE‐1 trial of lenabasum in diffuse cutaneous systemic sclerosis (dcSSc) allowed background immunosuppressive therapy (IST) at the discretion of individual investigators, and no significant differences were observed between treatment arms. This provides a powerful opportunity to compare the relative efficacy of different ISTs in a well‐defined large cohort of patients with dcSSc. Methods Prespecified IST categories, efficacy end points, baseline disease characteristics likely to influence efficacy outcomes, the definition of interstitial lung disease, definitions of IST use, and categories of IST use by which efficacy outcomes were evaluated were. Descriptive statistics are used to present results. Results For skin, change in modified Rodnan skin score (mRSS) was numerically greatest with mycophenolate mofetil (MMF) treatment in patients with the earliest disease, reaching −10.8 points in the MMF group versus −4.8 points in the no IST group in patients with a disease duration ≤2 years. Other ISTs had improvements intermediate between that seen in the MMF and no IST groups. Forced vital capacity (mL) was stable over 52 weeks in patients in the MMF group compared to an around 160‐mL decline over 52 weeks in no IST group. Differences in outcome were observed between antinuclear antibody subgroups, with greater difference in favor of MMF for skin and lungs being observed in anti–topoisomerase 1 autoantibody–positive patients. In contrast, anti–RNA polymerase III autoantibody–positive patients in both the no IST and MMF groups improved rapidly, with a decrease in mRSS. Conclusion Taken together, our findings robustly support routine use of MMF in dcSSc and show benefit especially in early‐stage disease. Those patients with high‐risk antibodies for lung fibrosis might be especially suitable for MMF treatment.

Background : To evaluate the use of hydroxychloroquine (HCQ) and its impact on Health Assessment Questionnaire disability index(HAQ-DI), the Cochin Hand Function Status(CHFS) in a large SSc cohort. Methods: SSc patients from the European Scleroderma Trials and Research (EUSTAR) database treated with HCQ for at least 6 months were evaluated and compared to a propensity matched group of SSc patients not using HCQ. Demographic and clinical data, concomitant drugs, HAQ-DI and CHFS (at least 2 evaluations) were recorded and were the outcome variables of interest. Statistical analysis was performed using propensity score matching for age, gender, disease duration, corticosteroids, immunosuppressives, vasoactive drugs in a 3:1 control:HCQ ratio. Standard descriptive statistics and Student’s T-test and Chi-square test were used to assess the propensity-matched groups. Results Out of 17,805 SSc patients evaluated, 468 (2.6%) constituted the HCQ group. Among them, 50 (10.7%) had at least a baseline and follow-up HAQ-DI evaluation and 44 (9.4%) had at least a baseline and follow-up CHFS evaluation. Propensity matching assured that patients were matched for female gender (HCQ vs control 92.0% vs. 85.3%), mean age (49.8 vs. 50.0 years) disease duration (8.3 vs. 9.1 years), limited disease (55.3 vs. 62.6%) as well as background medications (all P>0.1. We did not find any significant differences among the two groups in change of HAQ-DI CHFS, over 365 days (all P>0.05) Conclusions: Results from the EUSTAR registry showed that HCQ was used by 2.6% of SSc patients. HCQ use did not improve the HAQ-DI, or CHFS, comparing HCQ users to non-HCQ users

To investigate the diagnostic accuracy of lung ultrasound (LUS) for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). This retrospective study included patients over 18 years with RA evaluated at the Department of Rheumatology and Immunology of Shantou Central Hospital. All patients underwent chest high-resolution computed tomography (HRCT) and LUS within one month. The LUS was performed in a total of 50 scanning sites (ScS), and the number of B-lines present in each ScS was counted and summed up as B-lines score. A positive judgement was given on LUS when the B-lines score exceeded 10. The presence and patterns of ILD were defined by HRCT findings. ROC curve analysis was used to calculate the accuracy of LUS to detect ILD. A total of 120 RA patients (86 women, with a median age of 56.0 [50.0–64.0] years) were enrolled. Based on the HRCT, 76 patients were found to have radiographic ILD, with 63 exhibiting nonspecific interstitial pneumonia (NSIP) and 13 showing usual interstitial pneumonia (UIP). Sonographic ILD was detected in 76 patients who underwent LUS examination. The concordance rate between two modalities was 83.33

ObjectiveTwo randomized trials for patients with diffuse systemic sclerosis (SSc) demonstrated an overall survival (OS) and event-free survival (EFS) advantage of autologous hematopoietic stem cell transplantation (AHSCT) using CD34+ selected peripheral blood stem cells (PBSCs) compared with monthly cyclophosphamide (CY). We asked if an unmodified PBSC graft followed by maintenance mycophenolate mofetil (MMF) after AHSCT, instead of a CD34+ selected graft, could provide comparable AHSCT outcomes.MethodsTwenty patients with high-risk SSc were enrolled in a prospective, single-arm trial with CY 200 mg/kg and horse antithymocyte globulin (ATG; CY200/ATG), followed by unmanipulated autologous PBSC, and then MMF maintenance starting at 2 months after AHSCT.ResultsPoint estimates of OS and EFS at 5 years after AHSCT were 85% (95% confidence interval [CI] 60.4%-94.9%) and 75% (95% CI 50%-88.7%), respectively. Median follow-up was 7.5 years (range 5.6-11.6) after transplant for living patients. Eight patients (40%) required intensive care unit treatment early after transplant. Early transplant-related mortality occurred in two patients (10%). Five patients developed relapse/progression of SSc after AHSCT. Four of nine patients with anti-RNA polymerase III antibodies had prior scleroderma renal crisis and the lowest quartile of estimated glomerular filtration rate (eGFR) on study entry; all four patients developed prolonged organ failure/death early after transplant.ConclusionWe observed favorable OS and EFS after AHSCT for patients with SSc, using CY200/ATG, unmanipulated PBSCs, and MMF posttransplant maintenance, which was comparable to trials with CD34+ graft selection. We identified a possible risk factor, pretransplant low eGFR, for adverse outcomes after AHSCT.

Introduction: Digital ulcers are an important disease manifestation of systemic sclerosis and are associated with significant morbidity. As such, there is an urgent need for the development of evidence-based recommendations to guide clinicians in the treatment of digital ulcers.Methods: A steering committee of international experts was established. A systematic review of the literature pertaining to the use of pharmacologic treatments in the management of digital ulcers was performed to inform the development of treatment recommendations for systemic sclerosis digital ulcers. Consensus methodology was used to develop the final treatment recommendations.Results: The World Scleroderma Foundation committee agreed on 8 overarching treatment principles and 10 pharmacologic treatment recommendations for the management of systemic sclerosis digital ulcers. Phosphodiesterase 5 inhibitors and intravenous iloprost were recommended for the management of acute digital ulcers. Bosentan was recommended for prevention of digital ulcers.Conclusion: This study has yielded pragmatic treatment recommendations to direct treatment decisions for the management of systemic sclerosis digital ulcers. In addition, results have highlighted areas in need of future research in order to improve patient outcomes.