{"title":"Renal Manifestations in VEXAS Syndrome: A Systematic Review of Clinical Features, Pathology, and Outcomes.","authors":"Akhila Arya, Vishnu Mulavini, Rasiya Hashim, Madhumita Rondla, Jia Wei Tan, Dileep Unnikrishnan","doi":"10.31138/mjr.280525.apq","DOIUrl":"https://doi.org/10.31138/mjr.280525.apq","url":null,"abstract":"<p><strong>Objective: </strong>This review aimed to synthesise current literature on the clinical presentation, renal pathology, treatment, and outcomes of kidney involvement in VEXAS syndrome.</p><p><strong>Methods: </strong>We conducted a systematic review in accordance with PRISMA guidelines, including all original English-language publications from 2020 to May 14, 2025, that described any form of renal involvement in VEXAS syndrome. Data extracted included patient demographics, clinical presentation, renal biopsy findings, treatment strategies (including dialysis), clinical outcomes, and cause of death.</p><p><strong>Results: </strong>We included nine studies (five case series, three case reports, and one retrospective cohort), providing a total of 39 individual patient records. The reported frequency of renal involvement in VEXAS syndrome ranged from 3.6% to as high as 39% at 10-year follow-up. All documented cases involved male patients. The most common clinical presentations included acute kidney injury (AKI), chronic kidney disease (CKD), proteinuria, and haematuria. Among biopsy findings, interstitial nephritis was most frequently reported, followed by glomerulonephritis, minimal change disease, acute tubular injury, and IgA nephropathy. Less frequently, membranous nephropathy, peritubular capillaritis, glomerulosclerosis, and light chain cast nephropathy were also observed. Treatment primarily involved glucocorticoids, with steroid-sparing agents added based on the presence of coexisting non-renal manifestations. Renal outcomes were generally favourable, with most patients achieving recovery and only rare instances of dialysis dependence. The contribution of renal involvement to overall mortality remains unclear.</p><p><strong>Conclusion: </strong>Renal manifestations in VEXAS syndrome are varied but remain underexplored due to the rarity of the disease. Focused studies on kidney involvement are crucial to improve our understanding of the underlying pathophysiology and its clinical implications.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"110-116"},"PeriodicalIF":0.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145295/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843716","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":"Optimising Conventional Therapy in Rheumatoid Arthritis.","authors":"Alexandros A Drosos, Paraskevi V Voulgari","doi":"10.31138/mjr.311025.eti","DOIUrl":"https://doi.org/10.31138/mjr.311025.eti","url":null,"abstract":"<p><p>In the decades of the 1980s and 1990s, before the onset of biologic therapies for rheumatoid arthritis (RA) management, there was a significant number of patients who did not achieve remission or low disease activity (LDA), due to limited therapeutic choices. At that time, only some conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs), along with steroids, were used. Nowadays, despite the progress of RA management with the introduction of biologic (b) and targeted synthetic (ts) DMARDs, which showed significant clinical improvement and retardation of radiological damage, unmet needs of RA treatment still exist. On the other hand, the concept of treat-to-target (T2T) approach and tight control monitoring of disease activity, are considered now the cornerstone for achieving remission or LDA in RA patients. Several clinical trials confirmed the efficacy and safety of csDMARDs using the tight control and T2T strategies. Methotrexate is the fundamental drug for RA management and in combination with other csDMARDs, with or without steroids, it has proven to be efficacious and safe and less expensive in comparison to newer biologic therapies. Furthermore, MTX demonstrated cardioprotective effects and reduced the risk of cardiovascular events in RA patients Therefore, there is a potential for improving treatment strategies with conventional therapies in the management of early RA. Optimal and early use of csDMARDs controls disease activity similarly to biologic therapies and is less expensive.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"117-125"},"PeriodicalIF":0.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145289/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843698","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}
Arriana Gkouvi, Eleni C Pardali, Katerina Maria Kontouli, Eleni Patrikiou, Dimitrios P Bogdanos, Dimitrios G Goulis, Maria G Grammatikopoulou
{"title":"Translation and Validation of the Greek Version of the Revised Fibromyalgia Impact Questionnaire (FIQR).","authors":"Arriana Gkouvi, Eleni C Pardali, Katerina Maria Kontouli, Eleni Patrikiou, Dimitrios P Bogdanos, Dimitrios G Goulis, Maria G Grammatikopoulou","doi":"10.31138/mjr.190525.ria","DOIUrl":"https://doi.org/10.31138/mjr.190525.ria","url":null,"abstract":"<p><strong>Objective: </strong>Increasing awareness of chronic pain conditions, such as fibromyalgia, requires validated tools to monitor the impact of the disease and response to treatment. This study aimed to translate and validate the Revised Fibromyalgia Impact Questionnaire (FIQR) in Greek patients with fibromyalgia.</p><p><strong>Methods: </strong>The FIQR was translated into Greek (FIQR-GR) using the forward-backward-forward method. A total of 311 adults participated in this validation study by completing questionnaires and reporting their demographic characteristics (age, gender, weight, height, education, profession, and number of children). Internal consistency was evaluated using Cronbach's alpha, and confirmatory factor analysis (CFA) was conducted to assess the structural validity of the tool. Regression models were used to test the association between FIQR and demographic characteristics.</p><p><strong>Results: </strong>The FIQR-GR demonstrated excellent internal consistency (Cronbach's alpha=0.94, function domain 0.92, Overall Impact domain 0.85, symptoms domain 0.85). CFA revealed goodness of fit (CFI=0.900, RMSEA=0.080, SRMR=0.056) and confirmed the original three-factor model. Most items had high standardised factor loadings (0.51 to 0.86), providing strong evidence for validity, although two items had lower values than expected (0.40 for energy levels, 0.48 for depression). There was a significant positive association between FIQR and body mass index (BMI) (p<0.001), suggesting that patients with a higher BMI experienced more severe fibromyalgia symptoms.</p><p><strong>Conclusion: </strong>The FIQR-GR is a valid and reliable tool for Greek patients with fibromyalgia and a valuable tool in clinical and research settings.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"156-163"},"PeriodicalIF":0.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843731","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":"Comment on \"Methotrexate-Induced Accelerated Nodulosis: A Case Series\".","authors":"Pavlos Stamatis","doi":"10.31138/mjr.171125.voc","DOIUrl":"https://doi.org/10.31138/mjr.171125.voc","url":null,"abstract":"<p><p>Comment on \"Methotrexate-Induced Accelerated Nodulosis: A Case Series\" [Subramanian R et al, Mediterr J Rheumatol 2024;35(4):680-683].</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"273-274"},"PeriodicalIF":0.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145293/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843709","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":"Impairment of Diverse Patient-Reported Outcome Measures in Patients with Takayasu Arteritis: A Cohort Study.","authors":"Darpan R Thakare, Upendra Rathore, Kritika Singh, Tooba Qamar, Vikas Agarwal, Manas Ranjan Behera, Neeraj Jain, Manish Ora, Durga Prasanna Misra","doi":"10.31138/mjr.050625.qtr","DOIUrl":"https://doi.org/10.31138/mjr.050625.qtr","url":null,"abstract":"<p><strong>Aim: </strong>To comprehensively evaluate patient-reported outcome measures (PROMs) and fibromyalgia in patients with Takayasu arteritis (TAK) compared with healthy controls.</p><p><strong>Methods: </strong>Quality of life (QoL) using EuroQol-5D-3L (EQ-5D), disability using Health Assessment Questionnaire (HAQ), physical activity - Metabolic Equivalents of Task (METs) using International Physical Activity Questionnaire - Short Form (IPAQ-SF), work impairment using Work Productivity and Activity Impairment - General Health (WPAIGH), anxiety using Generalised Anxiety Disorder seven items (GAD-7), depression using Patient Health Questionnaire nine items (PHQ-9), fatigue using Multidisciplinary Fatigue Inventory (MFI) and Functional Assessment of Chronic Illness Therapy (FACIT) scales and fibromyalgia were assessed in patients with TAK. Comparisons were performed between PROMs in patients with TAK and age- and sex-similar healthy controls, TAK with active or inactive disease by physician global assessment (adjusted for Large Vessel Vasculitis Index of Damage scores), or the same patients with TAK over time.</p><p><strong>Results: </strong>Eighty-four patients with TAK (mean age 35.61 years, 61 females) were compared with 61 healthy controls (mean age 33.62 years, 45 females). Patients with TAK had worse QoL, disability, activity impairment due to health, anxiety, depression and fatigue than controls (p<0.01); similar proportions had fibromyalgia. After adjustment for damage scores, patients with active TAK had worse impairment of physical activity due to health and fatigue. PROMs remained stable on follow-up in the patients with TAK (mean follow-up interval 7.53 months).</p><p><strong>Conclusion: </strong>QoL, disability, anxiety, depression, and fatigue are impaired in patients with TAK and remain stable over time. Fatigue is associated with active TAK.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"164-178"},"PeriodicalIF":0.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145292/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843740","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":"https://doi.org/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":0.0,"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}
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":"https://doi.org/10.31138/mjr.230126.epr","url":null,"abstract":"","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"106-109"},"PeriodicalIF":0.0,"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}
{"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":"https://doi.org/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":0.0,"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}