Journal of Scleroderma and Related Disorders最新文献

{"title":"Palliative care in systemic sclerosis: incorporating advance care planning into longitudinal care.","authors":"Marissa B Savoie, Jessica L Logeman, Flavia V Castelino","doi":"10.1136/jsrd-2026-000020","DOIUrl":"10.1136/jsrd-2026-000020","url":null,"abstract":"<p><p>Despite advances in rheumatological treatments, patients with systemic sclerosis (SSc) suffer with myriad symptoms and experience associated quality of life decrements. Although SSc-related mortality is reported around 53%, there is a paucity of existing literature describing advance care planning and palliative care in SSc. Here we review symptom management burden and strategies by organ manifestation: interstitial lung disease, pulmonary hypertension, gastrointestinal involvement, cutaneous and musculoskeletal manifestations and digital ischaemia. Then we review domains of palliative care in the context of SSc including psychosocial support, sharing prognostic information, discussing goals of care, advance care planning and comfort-focused care. Finally we suggest unmet research needs in palliative care and SSc.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000020"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231730/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199044","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influence of comorbidities and patient demographics on Raynaud's symptom characteristics: implications for diagnosis and management.","authors":"Reshma Seomore, Matthew Wells, Ula Tymoszuk, Mithi Ahmed-Richards, Christopher P Denton, Francesco Del Galdo, Sue Farrington, Ariane L Herrick, Michael Hughes, Nick Jeffries-Owen, John D Pauling","doi":"10.1136/jsrd-2026-000008","DOIUrl":"10.1136/jsrd-2026-000008","url":null,"abstract":"<p><strong>Objectives: </strong>Associations between Raynaud's phenomenon (RP) and body mass index (BMI), fibromyalgia syndrome (FMS) and migraine have been reported but their influence on the lived experience of RP is unknown.</p><p><strong>Design: </strong>Cross-sectional electronic survey.</p><p><strong>Setting: </strong>A social media-based awareness campaign organised by Scleroderma & Raynaud's UK.</p><p><strong>Participants: </strong>4141 respondents with RP, including 3279 with primary RP (PRP), 476 with systemic sclerosis-related RP (SSc-RP) and 386 with other systemic autoimmune rheumatic disease-related RP (SARD-RP).</p><p><strong>Interventions: </strong>Not applicable.</p><p><strong>Main outcome measures: </strong>RP symptom characteristics (colour change patterns, pain, numbness, tingling and thumb involvement) and their associations with BMI, FMS and migraine.</p><p><strong>Results: </strong>In PRP, low BMI correlated with higher prevalence of cyanosis (55.1% vs 41.2%), hyperaemia (48.2% vs 41.3%), triphasic change (30.4% vs 23.0%) and thumb involvement (36.0% vs 27.0%) compared with high BMI. In PRP, concomitant FMS was associated with higher prevalence of pain (77.7% vs 57.4%), cyanosis (53.8% vs 45.4%), tingling (72.6% vs 62.8%) and thumb involvement (46.2% vs 27.2%) compared with those without FMS. Higher prevalence of pain was reported by FMS respondents with SSc-RP (82.8% vs 69.9%) and SARD-RP (84.1% and 70.9%, respectively). In PRP, migraine was associated with higher prevalence of cyanosis (50.0% vs 44.9%), hyperaemia (50.3% vs 43.7%), pain (69.0% vs 56.2%) and thumb involvement (33.4% vs 27.1%). Migraine was associated with higher prevalence of hyperaemia and pain in SARD-RP.</p><p><strong>Conclusion: </strong>BMI, FMS and migraine influence the lived experience of RP, particularly in PRP. FMS is associated with a greater pain burden, whereas low BMI and migraine are associated with prominent vasospastic features. These aetiopathogenic drivers influence RP symptomatology, with implications for management.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000008"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231726/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Knowledge and perceptions of medical practitioners on the role of nutrition in systemic sclerosis.","authors":"Elisavet Efthymiou, Eleni C Pardali, Arriana Gkouvi, Alexandros Mitropoulos, Christina G Katsiari, Eirini Koidou, Dimitrios G Goulis, Markos Klonizakis, Dimitrios P Bogdanos, Maria G Grammatikopoulou","doi":"10.1136/jsrd-2026-000005","DOIUrl":"10.1136/jsrd-2026-000005","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic 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.</p><p><strong>Methods: </strong>A 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.</p><p><strong>Results: </strong>Physicians 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%).</p><p><strong>Conclusion: </strong>Patients 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.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000005"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Scleroderma Clinical Trials Consortium (SCTC) outcome instruments for evaluation of systemic sclerosis.","authors":"Antonia Valenzuela, Lorinda Chung, John D Pauling, Robyn Domsic, Lesley Saketkoo, Kim Fligelstone, Anne-Marie Russell, Dinesh Khanna, Tracy Frech, Laura Ross, Mandana Nikpour, Christopher Denton, Murray Baron","doi":"10.1136/jsrd-2026-000009","DOIUrl":"10.1136/jsrd-2026-000009","url":null,"abstract":"<p><p>The Scleroderma Clinical Trials Consortium (SCTC) is committed to advancing the understanding and treatment of systemic sclerosis (SSc). This review focuses on the development and validation of various outcome instruments by the SCTC, which are crucial for evaluating the effectiveness of interventions in clinical trials and observational studies. Key instruments discussed include the Assessment of Systemic Sclerosis-Associated RAynaud's Phenomenon Questionnaire, the SCTC Radiologic Scoring System for calcinosis, the Mawdsley Questionnaire for calcinosis, the University of California Los Angeles SCTC Gastrointestinal Tract Instrument (GIT) 2.0, the SCTC Activity Index and the SCTC Damage Index. Additionally, the SCTC and World Scleroderma Foundation (WSF) Skin Scoring Certification is highlighted as an important resource for researchers. Each instrument's purpose, description and validation process are examined, underscoring their pivotal role in advancing SSc research and improving patient outcomes.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000009"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231724/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcriptomic profiling of myocardial biopsies reveals metabolic suppression and autophagy activation in systemic sclerosis-associated primary heart involvement.","authors":"Yohei Isomura, Yoshio Nakano, Yoshiaki Kubota, Tsunenori Saito, Satsuki Noma, Kai Miyazaki, Yukako Shintani-Domoto, Kuniya Asai, Satoru Miyazaki, Hayashi Yamamoto, Masataka Kuwana","doi":"10.1136/jsrd-2026-000003","DOIUrl":"10.1136/jsrd-2026-000003","url":null,"abstract":"<p><strong>Objectives: </strong>To characterise the myocardial transcriptomic landscape of patients with systemic sclerosis (SSc) with primary heart involvement (pHI) and identify molecular pathways underlying its pathogenesis.</p><p><strong>Design: </strong>A single-centre study exploring the molecular pathogenesis of SSc-pHI through transcriptomic analysis of endomyocardial biopsy specimens.</p><p><strong>Setting: </strong>Basic research.</p><p><strong>Participant: </strong>This study enrolled seven patients with SSc-pHI and eight patients with dilated cardiomyopathy (DCM) who had undergone endomyocardial biopsy for clinical practice purposes.</p><p><strong>Interventions: </strong>No intervention.</p><p><strong>Main outcome measures: </strong>Not applicable.</p><p><strong>Methods: </strong>Endomyocardial biopsy specimens from patients with SSc-pHI and those with DCM underwent whole RNA sequencing. To enable indirect comparison with non-failing (NF) myocardium, public RNA sequencing data of NF and DCM samples were integrated using DCM as a shared reference. Differential gene expression, pathway enrichment (Ingenuity Pathway Analysis and Gene Set Enrichment Analysis) and immune and stromal cell deconvolution were performed. Histopathological evaluation included LC3 immunostaining and transmission electron microscopy (TEM).</p><p><strong>Results: </strong>A total of 700 genes were differentially expressed between SSc and DCM myocardium. Mitochondrial energy metabolism pathways, including oxidative phosphorylation, fatty acid β-oxidation and the tricarboxylic acid cycle, were markedly suppressed in SSc. Indirect comparison with NF myocardium confirmed reciprocal regulation of mitochondrial metabolism and suggested enhanced autophagy. Cell deconvolution revealed enrichment of M1-like macrophages in SSc myocardium. LC3 immunostaining and TEM revealed increased autophagic vacuoles, lipid droplet accumulation and ischaemia-like ultrastructural alterations.</p><p><strong>Conclusions: </strong>SSc myocardium exhibits metabolic reprogramming characterised by mitochondrial dysfunction and enhanced autophagy, accompanied by macrophage activation.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000003"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231729/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"AhR agonist Ficz modulates T follicular helper cells and ameliorates skin fibrosis in a bleomycin-induced SSc mouse model.","authors":"Xianzhong Zhu, Yulin Chen, Chuangye Qiu, Maoyun Wei, Junxian Chen, Baoxuan Luo, Qing Qi","doi":"10.1136/jsrd-2026-000006","DOIUrl":"10.1136/jsrd-2026-000006","url":null,"abstract":"<p><strong>Objectives: </strong>T follicular helper (Tfh) cells are involved in the pathogenesis of systemic sclerosis (SSc). Recent investigations have revealed aryl hydrocarbon receptor (AhR) regulated skin fibrosis in SSc. This study aims to explore the effect of AhR agonist Ficz on Tfh in a bleomycin (BLM)-induced SSc mouse model.</p><p><strong>Methods: </strong>BALB/c mice were randomly assigned to the control group (Ctrl), BLM-induced SSc mouse group (BLM) and the Ficz treatment group (BLM+Ficz). BLM (1 mg/kg/day) and Ficz (1 µg/mouse/48 hours) were injected subcutaneously for 4 weeks. Skin lesions were harvested for HE staining, Masson's trichrome (Masson) staining, double immunofluorescence as well as real-time quantitative PCR. Flow cytometry analysis was carried out to determine the proportion of peripheral Tfh cells.</p><p><strong>Results: </strong>We found that Ficz treatment effectively attenuated dermal thickness, collagen content and the expression of fibrosis-related genes, including <i>Col1a1</i>, <i>Col3a1</i>, <i>Fn1</i> and <i>Tgfβ1</i> compared with the BLM-induced mice. Moreover, the percentage of peripheral Tfh cells (CD4+CXCR5+PD-1+) was significantly elevated in BLM-induced mice and Ficz appeared to reduce the percentage of peripheral Tfh cells. Double immunofluorescence staining verified the presence of Tfh-like cells (CXCR5+PD-1+) in skin lesions of BLM-induced mice. In addition, the messenger RNA level of a key Tfh cytokine, <i>Il21</i> was decreased in skin of Ficz treated BLM-induced mice, with elevated expression of <i>Cyp1a1</i>, a marker of AhR signalling.</p><p><strong>Conclusions: </strong>Ficz, an AhR agonist, can alleviate skin fibrosis in BLM-induced SSc mice, which may relate to the reduction of Tfh cells and its downstream cytokine interleukin-21 expression through the activation of AhR pathway.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000006"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231721/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Are we ready to predict progressive disease in juvenile systemic sclerosis?","authors":"Ivan Foeldvari, Jens Klotsche, Kathryn S Torok","doi":"10.1136/jsrd-2026-000011","DOIUrl":"10.1136/jsrd-2026-000011","url":null,"abstract":"","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000011"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231722/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systemic sclerosis and primary biliary cholangitis: a systematic review of case-control studies comparing isolated and overlapping disease phenotypes.","authors":"Meryem Nursoy, Esra Mehmetoglu, Amit Syal, Rishika Pasula, Konstantinos Parperis, Cuckoo Choudhary, Chris T Derk","doi":"10.1136/jsrd-2026-000002","DOIUrl":"10.1136/jsrd-2026-000002","url":null,"abstract":"<p><strong>Objectives: </strong>To explore variations in disease presentation between isolated systemic sclerosis (SSc), primary biliary cholangitis (PBC) and SSc-PBC overlap syndrome.</p><p><strong>Design: </strong>Systematic review conducted according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines.</p><p><strong>Setting: </strong>Literature search of PubMed, Scopus and Google Scholar for studies published through June 2025.</p><p><strong>Participants: </strong>13 studies (from 1331 initially identified records) including patients with isolated SSc, isolated PBC and SSc-PBC overlap were included. Eligible studies were case-control or cohort designs reporting demographic, serological or clinical outcomes.</p><p><strong>Interventions: </strong>Not applicable.</p><p><strong>Main outcome measures: </strong>Differences in clinical phenotype, serological markers, systemic involvement and mortality between SSc-PBC overlap and isolated disease groups.</p><p><strong>Results: </strong>Patients with SSc-PBC overlap demonstrated distinct clinical characteristics compared with isolated disease phenotypes. Anticentromere antibody and antimitochondrial antibody positivity were significantly higher in overlap patients. The overlap group predominantly included patients with limited cutaneous SSc and had a higher prevalence of extrahepatic autoimmune diseases, particularly Sjögren's syndrome and Hashimoto's thyroiditis. Systemic involvement, including pulmonary, musculoskeletal and cardiac manifestations, was less pronounced in overlap patients compared with isolated SSc. Liver-related mortality was higher in the PBC-only group, whereas mortality in the overlap group was primarily attributable to SSc-related causes.</p><p><strong>Conclusions: </strong>SSc-PBC overlap syndrome represents a distinct clinical and immunological entity. Recognition of this phenotype may facilitate earlier diagnosis, guide management strategies and improve patient outcomes.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000002"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231723/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199232","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"What is the treatment pattern of juvenile systemic sclerosis? Results from an international cohort.","authors":"Ivan Foeldvari, Kathryn Torok, Ozgur Kasapcopur, Amra Adrovic, Brian Feldman, Flavio Sztajnbok, Maria Teresa Terreri, Ana Paula Sakamoto, Sindhu Johnson, Jordi Anton, Valda Stanevicha, Archana Khan, Dieneke Schonenberg-Meinema, Eslam Al-Abadi, Ekaterina Alexeeva, Maria Katsicas, Sujata Sawhney, Vanessa Smith, Simone Appenzeller, Tadej Avcin, Mikhail Kostik, Thomas Lehman, Hana Malcova, Edoardo Marrani, Clare E Pain, Anjali Patwardhan, Walter-Alberto Sifuentes-Giraldo, Natalia Vasquez-Canizares, Patricia Costa-Reis, Mahesh Janarthanan, Monika Moll, Dana Nemcova, Maria Jose Santos, Sima Abu Al-Saoud, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Juergen Brunner, Despina Eleftheriou, Liora Harel, Gerd Horneff, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Siri Opsahl Hetlevik, Yosef Uziel, Jens Klotsche","doi":"10.1136/jsrd-2026-000010","DOIUrl":"10.1136/jsrd-2026-000010","url":null,"abstract":"<p><strong>Objectives: </strong>Clinical trial data are lacking for treatment of patients with juvenile systemic sclerosis (jSSc). Three published recommendations exist for jSSc but real-world data on treatment patterns are lacking. The aim of this study was to analyse treatments used in the jSSc inception cohort (jSSci) and compare to published recommendations on the treatment of jSSc.</p><p><strong>Methods: </strong>Data was extracted for patients with 24 months follow-up visits in the jSSci up until June 2023. Medications used and their association with clinical characteristics were analysed. Logistic regression analyses were performed to compare treatments between limited and diffuse cutaneous jSSc subtypes, organ involvement and time of initiation of treatment. Multilevel mixed effects logistic regression analyses were used to evaluate the change in medication use in follow-up. Treatment patterns were compared against published recommendations.</p><p><strong>Results: </strong>93 patients had 24 months follow-up data. 77% of patients were receiving disease-modifying treatment (DMARD) at enrolment, which increased to 91% at 24 months (p<0.001). Patients with diffuse cutaneous jSSc subtype had significantly more frequently active ulcerations, skin involvement and received any kind of treatment more often compared with limited (97% vs 91%, p=0.047). Methotrexate was used in 52% of patients at enrolment which decreased to 37% at 24 months (p=0.001). Mycophenolate mofetil use increased from 24% to 46% (p<0.001). Biological DMARDs increased from 5% to 22% (p<0.001). The treatment pattern strongly overlapped with the published paediatric guidance.</p><p><strong>Conclusion: </strong>This is the first report regarding the pattern of medication use in real life in the currently largest patient cohort of patients with jSSc. The observed pattern overlaps with the published recommendations.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000010"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231720/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictor factors of successful treatment response in interstitial lung disease-related systemic sclerosis.","authors":"Gantira Wijayakusumah Danasasmita, Suryo Anggoro Kusumo Wibowo, Cleopas Martin Rumende, Aulia Rizka, Sumariyono Sumariyono, Anna Ariane, Hamzah Shatri, Gurmeet Singh","doi":"10.1136/jsrd-2026-000012","DOIUrl":"10.1136/jsrd-2026-000012","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis (SSc) is a multi-organ autoimmune disease presenting interstitial lung disease (ILD) as the most common internal organ manifestation as it can be found in 25-90% of radiological imaging even without any symptoms. ILD in SSc (ILD-SSc) causes decreased mortality and morbidity, but the effectiveness of mycophenolate immunosuppression treatment reduces ILD progression by 75.5%. The risk of progression is still fairly high during immunosuppression, signifying the need to assess predictor factors for the success of mycophenolate treatment in patients with ILD-SSc.</p><p><strong>Objective: </strong>This study aims to investigate the incidence of successful mycophenolate treatment in ILD-SSc and predictor factors for the success based on real-world experience.</p><p><strong>Methods: </strong>A retrospective cohort design was applied in this study using outpatient medical record data of patients with extensive ILD-SSc from January 2021 to December 2024. Predictor model was developed from the results of multivariate analysis using linear regression followed by performance and internal validity tests.</p><p><strong>Results: </strong>A total of 119 patients with ILD-SSc who met the inclusion criteria were selected as study subjects. The incidence of successful mycophenolate treatment was 68.1%. Predictor factors influencing treatment success in ILD-SSc were obtained from multivariate analysis, such as younger age at onset of ILD-SSc has higher risk ratio for successfull treatment response (RR 2.327; 95% CI 1.510 to 3.586; p<0.001), type limited SSc (RR 0.389; 95% CI 0.251 to 0.602; p<0.001) and extent of ILD at initial diagnosis (RR 2.230; 95% CI 1.356 to 3.670; p=0.002). Predictor score model for mycophenolate treatment success with these three factors had an area under the curve performance of 0.671 (95% CI 0.567 to 0.776; p=0.001) and good calibration based on the Hosmer-Lemeshow test (p=0.576).</p><p><strong>Conclusion: </strong>The success rate of mycophenolate treatment in ILD-SSc at Dr. Cipto Mangunkusumo National Hospital (RSUPN) has comparable results with the reports in other countries. Factors such as age at onset of ILD-SSc diagnosis, extent of ILD and type of SSc are independent predictors of mycophenolate treatment success.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000012"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231719/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148199254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}