{"title":"Impact of Juvenile Dermatomyositis on Growth, Puberty, Bone Mineral Density, and Body Composition in Children.","authors":"Neha Sudhera, Harvinder Kaur, Vignesh Pandiarajan, Surjit Singh","doi":"10.31138/mjr.280725.ran","DOIUrl":"10.31138/mjr.280725.ran","url":null,"abstract":"<p><p>Juvenile Dermatomyositis (JDM) is a rare autoimmune disease characterised by chronic inflammation of the skeletal muscles and skin. This review examines effects of JDM and its treatments, particularly corticosteroids and immunosuppressive therapies, on growth, puberty, bone mineral density (BMD), and body composition. A comprehensive literature search was conducted in PubMed, EMBASE, Scopus, and Web of Science (1992-2025) using combinations of MeSH terms and keywords related to JDM, growth, puberty, BMD, fat distribution, lipodystrophy, and body composition. Methodological quality was appraised using a domain-based checklist adapted from the Newcastle-Ottawa Scale. High-dose corticosteroid therapy, which is commonly used to treat JDM, can lead to growth failure, delayed puberty, and changes in body composition, such as obesity, lipodystrophy, and fluctuations in muscle mass. Furthermore, long-term use of corticosteroids is linked to reduced BMD and an increased risk of osteoporosis and fractures. The chronic inflammation associated with JDM also contributes to metabolic complications, including insulin resistance, hyperlipidaemia, and heightened cardiovascular risk. Studies comparing JDM with other paediatric autoimmune conditions reveal that chronic disease activity and treatment significantly affect growth and pubertal development. However, much of the available evidence is derived from small, heterogeneous cohorts with inconsistent adjustment for factors such as pubertal status, nutrition, physical activity, and disease control. These limitations restrict interpretation and caution against overgeneralisation of findings. Standardised, adequately powered longitudinal studies are needed to clarify developmental trajectories and modifiers of risk. Clinically, proactive multidisciplinary care remains essential to optimise growth, bone health, and long-term metabolic outcomes in JDM.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"136-145"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145297/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843665","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}
Senol Kobak, Mehmet Hilmi Dogu, Mehmet Aydogan, Zehra Cagla Karakoc, Binnur Simsek
{"title":"VEXAS Syndrome and IgG4-Related Disease: Coincidental Association or Pathogenetic Link?","authors":"Senol Kobak, Mehmet Hilmi Dogu, Mehmet Aydogan, Zehra Cagla Karakoc, Binnur Simsek","doi":"10.31138/mjr.230126.epr","DOIUrl":"10.31138/mjr.230126.epr","url":null,"abstract":"","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"106-109"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843721","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}
Afnane Ismaili, Anas Chbihi, Ilyass Chergaoui, Anas Kherrab, Merieme Ghazi, Imane El Bouchti, Radouane Niamane
{"title":"Contribution of Trabecular Bone Score to Fracture Risk Stratification Beyond Bone Mineral Density in Women with Rheumatoid Arthritis: Results from a Cross-Sectional Cohort.","authors":"Afnane Ismaili, Anas Chbihi, Ilyass Chergaoui, Anas Kherrab, Merieme Ghazi, Imane El Bouchti, Radouane Niamane","doi":"10.31138/mjr.230725.rac","DOIUrl":"10.31138/mjr.230725.rac","url":null,"abstract":"<p><strong>Objective: </strong>To assess the contribution of trabecular bone score (TBS) to fracture risk stratification, in relation to bone mineral density (BMD) and vertebral fractures, in women with rheumatoid arthritis (RA).</p><p><strong>Methods: </strong>This monocentric cross-sectional study included 152 women with RA who underwent dual-energy X-ray absorptiometry (DXA) with TBS evaluation. BMD was measured at the lumbar spine and femoral neck. TBS values were categorised as normal (≥1.300), partially degraded (1.200-1.299), or degraded (≤1.200). Vertebral fractures were identified by vertebral fracture assessment (VFA). Crude and multivariate logistic regression analyses were performed to identify predictors of degraded TBS.</p><p><strong>Results: </strong>Based on BMD, 38.8% of patients were osteoporotic, while 69.1% had degraded TBS values. Vertebral fractures were detected in 30.3% of patients. Notably, 23.6% showed discordance, with TBS values more degraded than expected from their BMD category. In multivariate analysis, lower lumbar spine BMD (OR 3.62, 95% CI 1.67-7.83, p=0.001) and longer corticosteroid exposure (OR 1.87, 95% CI 1.01-3.45, p=0.046) were independently associated with degraded TBS.</p><p><strong>Conclusion: </strong>TBS provides complementary information to BMD in evaluating bone quality and fracture risk in RA. Its integration into clinical practice may improve fracture risk stratification in this high-risk population.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"206-211"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843707","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}
Sulaiman M Al-Mayouf, Ali AlAsmari, Alhanouf Al-Saleem
{"title":"Clinical Response to Baricitinib in Monogenic Lupus: Real-World Evidence from a Case Series of Seven Patients.","authors":"Sulaiman M Al-Mayouf, Ali AlAsmari, Alhanouf Al-Saleem","doi":"10.31138/mjr.160625.dsh","DOIUrl":"10.31138/mjr.160625.dsh","url":null,"abstract":"<p><strong>Background: </strong>Monogenic lupus is a rare, early-onset form of lupus caused by high-penetrance genetic variants, with diverse clinical features and a central role for type I interferon (IFN-I) in its pathogenesis. Standard treatments are lacking, but Janus kinase (JAK) inhibitors show promise, especially in refractory cases.</p><p><strong>Objective: </strong>To assess the safety and potential therapeutic benefits of baricitinib in children with monogenic lupus, based on real-world data from a single tertiary childhood lupus clinic.</p><p><strong>Methods: </strong>We conducted a retrospective observational study of patients with genetically confirmed monogenic treated with baricitinib between January 2023 and May 2025. Clinical, laboratory, and genetic data were analysed at baseline, six months, and the most recent follow-up. Outcomes included SLE Disease Activity Index (SLEDAI), Physician/Parent Global Assessment (GA), and daily glucocorticoid (GC) dose, and adverse events were collected.</p><p><strong>Results: </strong>Seven patients with mutations in <i>C1q, DNase1L3, PRKCD</i>, and <i>NRAS</i> were included. All had persistent disease activity despite multiple conventional and biologic therapies. Baricitinib was introduced as adjunct therapy. All patients tolerated baricitinib well, with no serious infections or adverse events reported. By six months, all patients showed improved disease activity, with SLEDAI scores decreasing from 14.2 to 4.1 by day 90 and remaining stable, PGA improving from 5.8 to 2.5 by day 180, and reduced daily GC doses.</p><p><strong>Conclusion: </strong>Baricitinib was well-tolerated and associated with clinical improvement in patients with treatment-refractory monogenic lupus. These findings support its potential therapeutic role and highlight the need for larger, long-term studies.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"186-192"},"PeriodicalIF":2.1,"publicationDate":"2026-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145278/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843663","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}
Chris T Derk, Ahmadreza Badali, Esra Mehmetoglu, Anvitha Mummadisetty, Amit Syal, Konstantinos Parperis
{"title":"Transcatheter Aortic Valve Replacement in Systemic Sclerosis: A Case Series and Review of Short- and Long-Term Outcomes.","authors":"Chris T Derk, Ahmadreza Badali, Esra Mehmetoglu, Anvitha Mummadisetty, Amit Syal, Konstantinos Parperis","doi":"10.31138/mjr.280525.ehr","DOIUrl":"10.31138/mjr.280525.ehr","url":null,"abstract":"<p><strong>Objectives: </strong>A higher frequency of valvular heart disease is seen among Systemic Sclerosis (SSc) patients. Advanced aortic valve stenosis leads to significant morbidity in these patients with multiple other comorbidities. In this study, we aim to define the short- and long-term outcomes of transcatheter aortic valve replacement (TAVR) procedures in SSc patients.</p><p><strong>Methods: </strong>We undertook a retrospective chart review of all patients with SSc who underwent a TAVR procedure at our institution over a defined 11-year period. Demographics as well as short- and long-term outcomes were identified.</p><p><strong>Results: </strong>Fourteen SSc patients underwent a TAVR procedure between 2012 and 2023. They were predominantly older Caucasian female patients with limited cutaneous SSc (lcSSc) with advanced aortic stenosis. Only one patient had a readmission within 30 days due to post-op heart failure and subsequently had to have the TAVR reversed to a SAVR.</p><p><strong>Conclusions: </strong>TAVR is a well-tolerated procedure in SSc patients with advanced aortic stenosis and multiple comorbidities.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"236-242"},"PeriodicalIF":2.1,"publicationDate":"2026-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145279/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843711","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":"Year in Review: Axial Spondyloarthritis.","authors":"Dimitrios Katsifis-Nezis, Antonis Fanouriakis","doi":"10.31138/mjr.300925.era","DOIUrl":"10.31138/mjr.300925.era","url":null,"abstract":"<p><p>Axial spondyloarthritis is a chronic inflammatory disease primarily affecting the sacroiliac joints and spine. Over the past two years, significant progress has been made across multiple domains of spondyloarthritis research. The current review critically summarises key advancements in research during 2024-2025, covering pathophysiology, epidemiology, diagnosis, imaging, and clinical management. Notable findings include novel immune stromal interactions, such as IL-17 extracellular vesicles, IL-33 induced TREM2<sup>+</sup> macrophages and disease specific CD8<sup>+</sup> T cell responses that provide insight into bone remodelling and inflammation. Epidemiological data confirm persistent diagnostic delays, though targeted referral strategies show promise. Advances in imaging include an international consensus on MRI protocols and the growing use of ultrasound for the assessment of enthesitis. Artificial intelligence models demonstrate strong performance in early diagnosis, imaging interpretation and disease activity prediction and prognosis, while advances in therapy include the first head-to-head trial and numerous real-world data.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 Suppl 1","pages":"1-11"},"PeriodicalIF":2.1,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12835917/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146094138","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}
Violeta Dimopoulou, Maria Nitsa, Christos Koutsianas
{"title":"Emerging Targets in Psoriatic Arthritis: Dual IL-17A/F and Selective TYK2 Inhibition in a Clinical Perspective.","authors":"Violeta Dimopoulou, Maria Nitsa, Christos Koutsianas","doi":"10.31138/mjr.101025.erh","DOIUrl":"10.31138/mjr.101025.erh","url":null,"abstract":"<p><p>Psoriatic arthritis (PsA) is a multifaceted immune-mediated inflammatory disease with several unmet therapeutic needs, and many patients fail to achieve comprehensive disease control with existing treatments. This has driven the exploration of novel therapeutic targets deriving from a better understanding of the immunopathogenetic cascade. Two of the most promising emerging targets are the dual inhibition of interleukin-17A and F (IL-17A/F) and the selective inhibition of tyrosine kinase 2 (TYK2). Bimekizumab, a monoclonal antibody that neutralises both IL-17A and IL-17F, has demonstrated superior efficacy over placebo and a standard-of-care TNF inhibitor in pivotal Phase III trials, achieving high rates of both joint and skin response. Its safety profile is characterised by a manageable increase in mild-to-moderate fungal infections, particularly oral candidiasis. Deucravacitinib, an oral, allosteric TYK2 inhibitor, represents a novel mechanism of action that modulates key cytokine pathways (IL-23, IL-12, Type I IFN) without inhibiting JAKs 1-3. Phase II and preliminary phase III data in PsA show significant improvements in joint and skin symptoms, with a safety profile from long-term psoriasis studies that appears favourable, showing minimal hematologic or laboratory abnormalities. The advent of bimekizumab and deucravacitinib enriches the PsA treatment arsenal and their introduction will help with more personalised management strategies for patients with PsA.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 Suppl 1","pages":"68-76"},"PeriodicalIF":2.1,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12869330/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146126962","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}
Konstantinos Kontoangelos, Charitomeni Vavouli, Ioanna Daikidou, Sofia Tsiori, Sofia Martinaki, Stavros Karageorgiou, Charalabos Papageorgiou, Alexander Stratigos
{"title":"Psychometric Evaluation of Patients with Psoriasis and their Association with Patients' Psychological Characteristics.","authors":"Konstantinos Kontoangelos, Charitomeni Vavouli, Ioanna Daikidou, Sofia Tsiori, Sofia Martinaki, Stavros Karageorgiou, Charalabos Papageorgiou, Alexander Stratigos","doi":"10.31138/mjr.030125.epr","DOIUrl":"10.31138/mjr.030125.epr","url":null,"abstract":"<p><strong>Objectives: </strong>The onset, progression and recurrence of psoriasis is believed to be related to mood and psychological disorders, such as depression. Psoriasis affects the personal, social and sexual life of patients resulting in psychological stress.</p><p><strong>Aim: </strong>The purpose of the research is the psychometric evaluation of patients with psoriasis.</p><p><strong>Methods: </strong>Seventy one patients with psoriasis were enrolled in the study. The measurement of the severity of psoriasis in the patients was implemented through specific indicators, the Psoriasis Area and Severity Index (PASI). Beck's Depression Inventory (BDI) was used to measure the intensity of depressive symptoms. In addition, the Eysenck Personality Questionnaire (EPQ), the self-completed scale Brief Symptom Inventory SCL - 90, and the HDHQ questionnaire (Psychometric Hostility and Direction of Hostility Questionnaire) were given. The validity and reliability of questionnaires rely on input from experts and potential responders who may suggest pertinent revisions to prepare forms with attractive designs, easily understandable questions, and correctly ordered points that appeal to target respondents.</p><p><strong>Results: </strong>Females with psoriasis have on average significantly higher scores in the BDI depression scale (13.5±10.0 vs. 7.9±8.3, p=0.009) compared to men, as well as in the SCL90 depression scale (13.64±10.18 vs. 7.00±5.45, p=0.003). The extroversion scale of the EPQ is statistically significantly related with the patients' psychiatric history and stable income. Patients receiving medication for psoriasis are 2 times more likely to answer the lie questions positively than patients not receiving medication (OR=2.01, p=0.028).</p><p><strong>Conclusion: </strong>Psoriasis exerts a direct influence on the social daily life of the individual, at a functional and behavioural level. It is essential to emphasise the importance of addressing the psychological effects of psoriasis along with its physical aspects for better outcomes.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 Suppl 1","pages":"92-99"},"PeriodicalIF":2.1,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12835929/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146094140","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}