Mediterranean Journal of Rheumatology最新文献

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Extensive Subcutaneous Rheumatoid Nodules Reflecting High Burden of Disease Activity. 广泛的皮下类风湿结节反映疾病活动的高负担。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-04-21 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.200625.rha
Georgios A Drosos, Anastasia K Zikou, Paraskevi V Voulgari, Alexandros A Drosos
{"title":"Extensive Subcutaneous Rheumatoid Nodules Reflecting High Burden of Disease Activity.","authors":"Georgios A Drosos, Anastasia K Zikou, Paraskevi V Voulgari, Alexandros A Drosos","doi":"10.31138/mjr.200625.rha","DOIUrl":"10.31138/mjr.200625.rha","url":null,"abstract":"<p><p>Rheumatoid arthritis (RA) is a chronic autoimmune systemic disease affecting mostly the small joints of the hands and feet. Extra-articular manifestations (EAMs) comprise the involvement of the skin, eyes, heart and lung. Among them, subcutaneous rheumatoid nodules (RN) are a common manifestation of systemic disease. However, disorders such as rheumatoid nodulosis, multicentric reticulohistocytosis (MRH) and others present subcutaneous nodules, similar to those seen in RA patients. A 41-year-old woman with a 10-year history of untreated RA, presented complaining of pain and tenderness in the small joints of the hands and feet. Past medical and family history were unremarkable. She presented swelling and tenderness on all metacarpophalangeal joints (MCPs) bilaterally and had multiple widespread subcutaneous RN affecting almost all the fingers of the hands, as well as, the Achille's tendons in a symmetrical manner. She had elevated levels of acute phase reactants and high titres of autoantibodies. Hand x-rays revealed severe erosive changes affecting all MCPs joints bilaterally. Thus, in this review we discuss the differential diagnosis of subcutaneous RN and their role in RA disease activity. This is an educational case of a patient with RA and extensive subcutaneous RN affecting the upper and lower extremities. To this end, physicians must be familiar and recognise early these signs which reflect RA disease activity and treat appropriately.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"427-430"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305904/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346464","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}
引用次数: 0
Inflammation at the Crossroads: Familial Mediterranean Fever and Cardiovascular Risk. 十字路口的炎症:家族性地中海热和心血管风险。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-04-21 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.121025.fcr
Sejla Karup, Sura Nur Baspinar, Batuhan Ayci, Feyza Nur Azman Tunc, Alperen Akyel, Yelin Guler, Serdal Ugurlu
{"title":"Inflammation at the Crossroads: Familial Mediterranean Fever and Cardiovascular Risk.","authors":"Sejla Karup, Sura Nur Baspinar, Batuhan Ayci, Feyza Nur Azman Tunc, Alperen Akyel, Yelin Guler, Serdal Ugurlu","doi":"10.31138/mjr.121025.fcr","DOIUrl":"10.31138/mjr.121025.fcr","url":null,"abstract":"<p><strong>Background: </strong>The understanding between Familial Mediterranean Fever (FMF) and cardiovascular diseases (CVDs) remains unclear. The study's objective was to investigate the association between FMF and CVD.</p><p><strong>Methods: </strong>Based on a survey targeting individuals with FMF, the patients diagnosed with coronary artery disease, cerebrovascular disease, and hypertension were considered patients with CVD.</p><p><strong>Results: </strong>Out of 522 patients, 201 had CVD. The mean ± standard deviation (SD) age was 53.00 ± 6.43 in patients without CVD and 57.60 ± 8.33 in patients with CVD. Hypertension was present in 188 patients (36%), coronary artery disease was observed in 51 patients (9.8%), and 10 patients (1.9%) had cerebrovascular disease. In patients with CVD, diabetes (30.3%) was the most common comorbidity. All patients consumed colchicine. Thirty-five patients with (17.2%) and 25 without CVD (7.8%) had colchicine resistance. 90% continued treatment with colchicine. 25 (12.4%) patients with CVD were using biological agents, as were 18 (5.7%) patients without CVD (p=0.006). The attack period average C-reactive protein (CRP) level was 61.3 (SD=53.1), while in remission, it had an average value of 3.39 (SD=4.28). CRP median was 3.00 (3.65) during attacks in the group with CVD, and 2.00 (2.5) in the group without CVD (p=0.011). CRP median was 2.8 (3.55) in patients with hypertension, and 2.00 (2.7) in patients without hypertension.</p><p><strong>Conclusion: </strong>FMF does not appear to increase the risk of CVD. Colchicine resistance was associated with the incidence of cardiovascular diseases. Colchicine and anti-interleukin-1 show promise in reducing the risk of cardiovascular diseases in patients with FMF.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"415-420"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305840/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346424","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}
引用次数: 0
Real-World Effectiveness of Baricitinib in Rheumatoid Arthritis: Significant Reduction of Disease Activity Independent of Age in a Multicentre Retrospective Cohort Study. Baricitinib治疗类风湿性关节炎的实际疗效:在一项多中心回顾性队列研究中,与年龄无关的疾病活动性显著降低。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-04-21 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.110525.ain
Daniele Lini, Matteo Colina, Romina Andracco, Francesca Ometto, Viviana Ravagnani, Alberto Lo Gullo, Simone Parisi, Antonella Farina, Patrizia Del Medico, Marino Paroli, Olga Addimanda, Alessandra Bezzi, Federica Lumetti, Aldo Biagio Molica Colella, Davide Murgia, Maddalena Larosa, Aurora Ianniello, Elena Bravi, Marta Priora, Palma Scolieri, Alessandro Volpe, Francesco Girelli, Elisa Visalli, Alarico Ariani, Eleonora Celletti, Bernd Raffeiner, Veronica Franchina, Rosalba Caccavale, Francesca Serale, Valeria Nucera, Cecilia Giampietro, Riccardo Bixio, Guido Rovera, Alessia Fiorenza, Maria Chiara Ditto, Ilaria Platè, Fabio Mascella, Eleonora Di Donato, Adorni Giuditta, Daniele Santilli, Natalia Mansueto, Gianluca Smerilli, Giulio Ferrero, Amati Gabriele, Francesco Molica Colella, Giorgio Amato, Francesco De Lucia, Ylenia Dal Bosco, Roberta Foti, Simone Bernardi, Myriam Di Penta, Emanuela Sabatini, Francesco Cipollone, Gilda Sandri, Rosario Foti, Gerolamo Bianchi, Maria Cristina Focherini, Vincenzo Bruzzese, Gianluca Lucchini, Eugenio Arrigoni, Rosetta Vitetta, Dario Camellino, Antonio Marchetta, Enrico Fusaro, Massimo Reta, Dilia Giuggioli, Andrea Becciolini
{"title":"Real-World Effectiveness of Baricitinib in Rheumatoid Arthritis: Significant Reduction of Disease Activity Independent of Age in a Multicentre Retrospective Cohort Study.","authors":"Daniele Lini, Matteo Colina, Romina Andracco, Francesca Ometto, Viviana Ravagnani, Alberto Lo Gullo, Simone Parisi, Antonella Farina, Patrizia Del Medico, Marino Paroli, Olga Addimanda, Alessandra Bezzi, Federica Lumetti, Aldo Biagio Molica Colella, Davide Murgia, Maddalena Larosa, Aurora Ianniello, Elena Bravi, Marta Priora, Palma Scolieri, Alessandro Volpe, Francesco Girelli, Elisa Visalli, Alarico Ariani, Eleonora Celletti, Bernd Raffeiner, Veronica Franchina, Rosalba Caccavale, Francesca Serale, Valeria Nucera, Cecilia Giampietro, Riccardo Bixio, Guido Rovera, Alessia Fiorenza, Maria Chiara Ditto, Ilaria Platè, Fabio Mascella, Eleonora Di Donato, Adorni Giuditta, Daniele Santilli, Natalia Mansueto, Gianluca Smerilli, Giulio Ferrero, Amati Gabriele, Francesco Molica Colella, Giorgio Amato, Francesco De Lucia, Ylenia Dal Bosco, Roberta Foti, Simone Bernardi, Myriam Di Penta, Emanuela Sabatini, Francesco Cipollone, Gilda Sandri, Rosario Foti, Gerolamo Bianchi, Maria Cristina Focherini, Vincenzo Bruzzese, Gianluca Lucchini, Eugenio Arrigoni, Rosetta Vitetta, Dario Camellino, Antonio Marchetta, Enrico Fusaro, Massimo Reta, Dilia Giuggioli, Andrea Becciolini","doi":"10.31138/mjr.110525.ain","DOIUrl":"10.31138/mjr.110525.ain","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the real-world effectiveness of baricitinib (BARI) in rheumatoid arthritis (RA), compare outcomes in patients <65 years versus ≥65 years, and identify predictors of DAS28-ESR remission/low disease activity (LDA) at 6 and 12 months.</p><p><strong>Methods: </strong>Retrospective multicentre cohort (n=423). Baseline variables included age, sex, disease duration, and comorbidities. DAS28-ESR was recorded at baseline, 6 and 12 months; remission was defined as DAS28-ESR <2.6 and LDA as ≤3.2. Predictors of remission/LDA were assessed with multivariable logistic regression, reporting odds ratios (ORs), 95% confidence intervals (CIs), and p-values.</p><p><strong>Results: </strong>Median age was 60 years (IQR 50.5-69.5); 79% were female; median disease duration 76 months (IQR 31-157). Compared with patients <65, those ≥65 had longer disease duration (94 vs 65 months; p<0.05) and more diabetes (16% vs 5%), dyslipidaemia (47% vs 19%), and hypertension (62% vs 33%) (all p<0.05). Median DAS28-ESR fell from 5.42 (IQR 4.84-6.06) at baseline to 3.71 (2.83-4.59) at 6 months and 3.29 (2.42-4.19) at 12 months (global repeated-measures test significant). Remission+LDA was achieved by 135/421 (32.1%) at 6 months and 182/423 (43.0%) at 12 months; remission alone increased from 65/421 (15.4%) to 117/423 (27.7%). Age ≥65 was not associated with response (6 months: OR 0.71, 95% CI 0.44-1.14, p=0.16; 12 months: OR 0.83, 95% CI 0.55-1.24, p=0.37). ACPA positivity independently predicted remission/LDA (6 months: OR 2.32, 95% CI 1.43-3.77, p<0.05; 12 months: OR 1.71, 95% CI 1.12-2.59, p<0.05). Prior JAK inhibitor exposure was not associated with reduced response. Results were consistent in a per-protocol sensitivity analysis.</p><p><strong>Conclusion: </strong>In this large multicentre real-world cohort, BARI significantly reduced disease activity over 12 months, with comparable effectiveness across age groups. ACPA positivity emerged as an independent predictor of achieving remission/LDA, supporting its potential role in treatment stratification.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"336-346"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346554","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}
引用次数: 0
Phenotypic Heterogeneity in Familial Takayasu Arteritis Across Two Families: A Case-Based Review. 家族性高须动脉炎在两个家族中的表型异质性:一项基于病例的回顾。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-04-21 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.120825.atf
Anisio U L Santana, Samuel K Shinjo
{"title":"Phenotypic Heterogeneity in Familial Takayasu Arteritis Across Two Families: A Case-Based Review.","authors":"Anisio U L Santana, Samuel K Shinjo","doi":"10.31138/mjr.120825.atf","DOIUrl":"10.31138/mjr.120825.atf","url":null,"abstract":"<p><strong>Background: </strong>Takayasu arteritis (TAK) is a rare large-vessel vasculitis that predominantly affects young women. Familial cases are uncommon but suggest a possible genetic predisposition.</p><p><strong>Case report: </strong>We describe five familial TAK cases from two families. In the first, two sisters had distinct disease patterns: one with severe supra-aortic involvement (Hata IIa), requiring intensive immunosuppression and vascular interventions; the other with isolated abdominal aortic disease (Hata IV), successfully managed with standard therapy. In the second family, three siblings presented with varying severity. One sister had mild disease responsive to therapy, whereas another developed rapidly progressive TAK with multivessel involvement, necessitating complex surgical management and ultimately succumbing to COVID-19 complications. Their brother presented with extensive Hata V involvement and achieved remission following brief medical therapy.</p><p><strong>Conclusion: </strong>Familial TAK demonstrates marked clinical and radiological heterogeneity, even among first-degree relatives. These findings underscore the importance of clinical vigilance and prompt evaluation for relatives who develop suggestive symptoms, to enable timely diagnosis and improve clinical outcomes.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"421-426"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346529","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}
引用次数: 0
Positive Effects of Conscious Connected Breathing Exercises on Sexual Function, Sleep, and Mood in Women with Fibromyalgia Syndrome Compared to Controls. 与对照组相比,有意识连接呼吸练习对纤维肌痛综合征女性性功能、睡眠和情绪的积极影响
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-04-21 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.080925.ers
Gulsum Uysal, Gulşah Yaşa Ozturk, Burhan Fatih Kocyiğit, Halenur Colak
{"title":"Positive Effects of Conscious Connected Breathing Exercises on Sexual Function, Sleep, and Mood in Women with Fibromyalgia Syndrome Compared to Controls.","authors":"Gulsum Uysal, Gulşah Yaşa Ozturk, Burhan Fatih Kocyiğit, Halenur Colak","doi":"10.31138/mjr.080925.ers","DOIUrl":"10.31138/mjr.080925.ers","url":null,"abstract":"<p><strong>Aim: </strong>Fibromyalgia syndrome (FMS) is a chronic rheumatic disorder characterised by body pain, decreased pain threshold and psychological distress. Our aim is to investigate the effects of breathing exercises on sexual function, sleep, and mood in FMS.</p><p><strong>Methods: </strong>This is a cross-sectional study comparing psychological assessments conducted prior to and following breathing exercises. Participants completed the Fibromyalgia Impact Questionnaire (FIQ), Hospital Anxiety and Depression Scale (HADS), and Jenkins Sleep Rating Scale (JSS) and Female Sexual Function Index (FSFI). Scores were analysed for comparison to controls.</p><p><strong>Results: </strong>A total of 56 women were enrolled to the study (30 intervention group, 26 controls). The mean age was 50.79 ± 9.06 years in intervention group. There were no statistical difference between groups occupational status (p > 0.05). The FIQ, HADS-depression, HADS anxiety, JSS and FSFI scores were similar between groups before breathing exercises (p > 0.05). FIQ, HADS depression- HADS anxiety, JSS scores after breathing exercises were better comparing to controls, p<0.001, p<0.001, p=0.002 and p=0.001, respectively. Although all symptoms were alleviated, the FSFI scores were not higher than FSD (Female sexual dysfunction) cut off value in intervention group after breathing exercises (p=0.033). The total FSFI score less than 26.55 was the cut-off for FSD. All of the participants had FSD.</p><p><strong>Conclusion: </strong>Breathing exercises can positively influence quality of sleep, anxiety, depression, and sexual life in patients with FMS. Incorporating breathing exercises into the curriculum may be considered to balance the quality of life for women with FMS.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"399-405"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305838/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346540","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}
引用次数: 0
Serum Sclerostin Correlates with ACPA Positivity in Post-Menopausal Women with Rheumatoid Arthritis: Data from a Cross-Sectional Case-Control Study in Greece. 绝经后类风湿关节炎妇女血清硬化蛋白与ACPA阳性相关:来自希腊横断面病例对照研究的数据
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-03-27 eCollection Date: 2026-06-01 DOI: 10.31138/mjr.190425.eda
Michail I Krikelis, Dimitra Moschou, Konstantina Zoupidou, Evangelia Mole, Konstantinos Makris, Simeon Tournis, Sousana Gazi, Vassilios Nikolaou, Clio Mavragani, Efstathios Chronopoulos
{"title":"Serum Sclerostin Correlates with ACPA Positivity in Post-Menopausal Women with Rheumatoid Arthritis: Data from a Cross-Sectional Case-Control Study in Greece.","authors":"Michail I Krikelis, Dimitra Moschou, Konstantina Zoupidou, Evangelia Mole, Konstantinos Makris, Simeon Tournis, Sousana Gazi, Vassilios Nikolaou, Clio Mavragani, Efstathios Chronopoulos","doi":"10.31138/mjr.190425.eda","DOIUrl":"10.31138/mjr.190425.eda","url":null,"abstract":"<p><strong>Introduction: </strong>Rheumatoid arthritis (RA) is a chronic inflammatory disease marked by bone loss, partly due to impaired Wnt signalling. The role of sclerostin, an endogenous Wnt inhibitor, in RA remains unclear.</p><p><strong>Methods: </strong>This 9-month cross-sectional case-control study included post-menopausal women recruited at a tertiary hospital in Athens, Greece. Participants underwent clinical assessment, DXA, radiographs, and blood tests. The aim was to assess serum sclerostin levels and their associations in RA.</p><p><strong>Results: </strong>A total of 97 RA patients and 45 controls were included. Mean age was 67 ± 7 years; median RA duration was 8 years; 40% were seropositive; 65% received bDMARDs. Median sclerostin levels did not differ significantly between groups (p=0.645). In RA patients, sclerostin levels were positively associated with ACPA (p=0.043). Notably, 27% of RA patients had extremely high sclerostin levels versus 11% of controls (p=0.035). Within this subgroup, high sclerostin was inversely associated with sarcopenia (p=0.020).</p><p><strong>Discussion: </strong>Although overall sclerostin levels were similar between groups, higher levels in seropositive RA suggest a possible link with ACPA.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 2","pages":"347-356"},"PeriodicalIF":2.1,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346267","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}
引用次数: 0
Mononeuritis Multiplex Presenting with Retroperitoneal Inflammation: A Case-Based Review in the Light of Diagnostic and Therapeutic Challenges. 以腹膜后炎症为表现的多发性单神经炎:基于诊断和治疗挑战的病例回顾。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-03-01 DOI: 10.31138/mjr.280725.ecz
Michail-Angelos Mourtzos, Natalia-Maria Keklikoglou, Dimitrios I Patoulias
{"title":"Mononeuritis Multiplex Presenting with Retroperitoneal Inflammation: A Case-Based Review in the Light of Diagnostic and Therapeutic Challenges.","authors":"Michail-Angelos Mourtzos, Natalia-Maria Keklikoglou, Dimitrios I Patoulias","doi":"10.31138/mjr.280725.ecz","DOIUrl":"10.31138/mjr.280725.ecz","url":null,"abstract":"<p><strong>Background: </strong>Mononeuritis multiplex (MNM), is an uncommon, peripheral neuropathy, presenting with asymmetric involvement of multiple individual nerves. It is frequently associated with vasculitic disorders and other immune-related conditions. Early manifestations can often be non-specific, which might complicate diagnosis.</p><p><strong>Case presentation: </strong>A 56-year-old woman with no significant medical history developed sudden abdominal and right gluteal pain followed by progressive and asymmetric sensorimotor deficits. Initial imaging demonstrated fat stranding and infiltration in the retroperitoneum, lateral pelvic walls, and lumbosacral plexus, suggestive of an inflammatory process. Laboratory testing demonstrated marked leucocytosis, eosinophilia, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), sterile pyuria, hypoalbuminemia and elevated alpha- and beta-globulins. Markers of infectious and autoimmune diseases, as well as neoplastic markers were unrevealing. Nerve biopsy was inconclusive. Therefore, a diagnosis of immune-mediated vasculitic neuropathy was favoured but not confirmed. She was treated with high-dose oral corticosteroids tapered over 12 months, with additional antibiotics to reduce risk of infection. At follow-up, she remained ambulatory and independent, with mild residual deficits and intermittent inflammatory marker elevation.</p><p><strong>Conclusions: </strong>This case illustrates how MNM presenting with gradual evolution, mixed infectious and autoimmune features, and in the context of retroperitoneal inflammation, can complicate the diagnostic process. In absence of biopsy confirmation, management required balancing empirical immunosuppression with infection risk. Comparison with prior reports underlines the importance of complete autoimmune workup, biopsy and imaging techniques such as positron emission tomography scan in minimising diagnostic uncertainty.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"212-219"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843680","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}
引用次数: 0
Immunoglobulin-G4 Related Disease: A Rare Entity with Many Clinical Faces - Literature Review and Case Illustration. 免疫球蛋白g4相关疾病:具有许多临床特征的罕见疾病-文献回顾和病例说明。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-03-01 DOI: 10.31138/mjr.150825.era
Vijay Krishnan R, Hariharan Seshadri, Suganya Balachandran, V Aribalaji, Umadevi T B
{"title":"Immunoglobulin-G4 Related Disease: A Rare Entity with Many Clinical Faces - Literature Review and Case Illustration.","authors":"Vijay Krishnan R, Hariharan Seshadri, Suganya Balachandran, V Aribalaji, Umadevi T B","doi":"10.31138/mjr.150825.era","DOIUrl":"10.31138/mjr.150825.era","url":null,"abstract":"<p><strong>Introduction: </strong>Immunoglobulin-G4 Related Disease (IgG4-RD) is a group of multi-system, fibro-inflammatory conditions characterised by elevated IgG4 levels and unique histopathological features. Clinical presentations of the disease are highly variable, albeit there exist distinct phenotypes in the presentations of the disease. IgG4-RD is a differential diagnosis that should be missed in any case of chronic inflammatory and tumefactive pathology.</p><p><strong>Case discussion: </strong>We present the case of a 47-year-old diabetic female who presented with features suggestive of acute pyelonephritis. On further evaluation, our findings unmasked a rare constellation of pancreatitis, orbital pseudotumor, and venous thrombosis. After an astute clinical observation by our team, we were able to decipher these as features of distinct phenotypes under the IgG4-RD spectrum with minimal overlap (pancreato-hepatobiliary disease, head and neck disease, and retroperitoneal disease groups, respectively).</p><p><strong>Literature review: </strong>We have presented an extensive review of the recent literature pertaining to the etiopathogenesis, clinical features, diagnostic protocol and management consensus for IgG4-related disease. A detailed account of the possible clinical presentations and work-up strategy for suspected patients has been emphasised in particular.</p><p><strong>Conclusion: </strong>IgG4-RD is diverse clinico-pathological entity that warrants a high degree of clinical suspicion. Once identified, efforts must be made to screen all possible foci of disease activity characteristic to the disease, as a multi-focal disease presentation of IgG4-RD is not unlikely.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"126-135"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145280/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843735","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}
引用次数: 0
Real-World Effectiveness of MEpolizuMaB in Eosinophilic Granulomatosis with Polyangiitis in Greece: A Protocol for a Multicentre Retrospective Observational Study - The MEMBER Study. MEpolizuMaB治疗希腊嗜酸性肉芽肿合并多血管炎的实际疗效:一项多中心回顾性观察性研究的方案-成员研究。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-03-01 DOI: 10.31138/mjr.070825.arn
Christos Koutsianas, Katerina Antoniou, Dimitrios Boumpas, Theodoros Dimitroulas, Christina G Katsiari, Menelaos Manoussakis, Kostantinos Porpodis, Kostantinos Samitas, Paschalis Steiropoulos, Paraskevi V Voulgari, Zafeiris Louvaris, Dimitrios Vassilopoulos
{"title":"Real-World Effectiveness of MEpolizuMaB in Eosinophilic Granulomatosis with Polyangiitis in Greece: A Protocol for a Multicentre Retrospective Observational Study - The MEMBER Study.","authors":"Christos Koutsianas, Katerina Antoniou, Dimitrios Boumpas, Theodoros Dimitroulas, Christina G Katsiari, Menelaos Manoussakis, Kostantinos Porpodis, Kostantinos Samitas, Paschalis Steiropoulos, Paraskevi V Voulgari, Zafeiris Louvaris, Dimitrios Vassilopoulos","doi":"10.31138/mjr.070825.arn","DOIUrl":"10.31138/mjr.070825.arn","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis occurring predominantly in patients with eosinophilic asthma. Glucocorticoids (GCs) are the cornerstone of treatment for EGPA to achieve disease remission, however, relapses despite GCs therapy are common. Mepolizumab (Nucala<sup>®</sup>) is a humanised monoclonal antibody that binds interleukin (IL)-5 and randomised controlled trials have shown that it effectively targets eosinophil-driven manifestations of EGPA. Since the vasculitic component of EGPA may not be solely driven by eosinophils, the aim of this retrospective observational study is to use real-world evidence to assess clinical outcomes following treatment with mepolizumab (Nucala<sup>®</sup>). A retrospective, observational, cohort study will be conducted in 10 hospital centres specialised in the treatment and monitoring of patients with EGPA in Greece. Data will be retrospectively collected and analysed from medical records of 70 patients with EGPA treated with mepolizumab (Nucala<sup>®</sup>) for ≥ 6 months, covering the period from September, 2021, to December, 2024.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"251-257"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843746","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}
引用次数: 0
Predictors of Cyclophosphamide Resistance in Granulomatosis with Polyangiitis: A Retrospective Cohort Study. 肉芽肿合并多血管炎患者环磷酰胺耐药性的预测因素:一项回顾性队列研究。
IF 2.1
Mediterranean Journal of Rheumatology Pub Date : 2026-03-01 DOI: 10.31138/mjr.150736.pcr
Pınar Akyüz Dağlı, Esra Kayacan Erdoğan, Hatice Ecem Konak, Bahar Özdemir Ulusoy, Serdar Can Güven, Berkan Armağan, Rezan Koçak Ulucaköy, Kevser Orhan, Ebru Atalar, İsmail Doğan, Yüksel Maraş, Ahmet Omma, Orhan Küçükşahin, Şükran Erten, Hakan Babaoğlu
{"title":"Predictors of Cyclophosphamide Resistance in Granulomatosis with Polyangiitis: A Retrospective Cohort Study.","authors":"Pınar Akyüz Dağlı, Esra Kayacan Erdoğan, Hatice Ecem Konak, Bahar Özdemir Ulusoy, Serdar Can Güven, Berkan Armağan, Rezan Koçak Ulucaköy, Kevser Orhan, Ebru Atalar, İsmail Doğan, Yüksel Maraş, Ahmet Omma, Orhan Küçükşahin, Şükran Erten, Hakan Babaoğlu","doi":"10.31138/mjr.150736.pcr","DOIUrl":"10.31138/mjr.150736.pcr","url":null,"abstract":"<p><strong>Objective: </strong>Cyclophosphamide remains a cornerstone of remission induction therapy in granulomatosis with polyangiitis (GPA); however, the presence of resistant patients necessitating a switch to alternative agents, such as rituximab, remains a clinical challenge. Identifying predictors of cyclophosphamide resistance could improve patient stratification and optimise treatment strategies.</p><p><strong>Methods: </strong>This retrospective cohort study included 75 patients diagnosed with GPA and treated at Ankara Bilkent City Hospital between 2018 and 2023.Clinical and laboratory data were extracted from electronic medical records. Baseline characteristics, organ involvement, and serological profiles were compared between cyclophosphamide-responsive and cyclophosphamide-resistant patients. Logistic regression analysis was performed to identify independent predictors of treatment resistance.</p><p><strong>Results: </strong>75 patients with GPA analysed. The mean age was 46.1years (SD=14.0).56 patients received cyclophosphamide as first-line therapy, of whom 34 (60.7%) achieved remission, while 22 (39.3%) switched to rituximab. Of these, 17 patients (30.4%) were classified as cyclophosphamide-resistant, while 5 patients (8.9%) switched due to other reasons. Younger age was a significant predictor of cyclophosphamide resistance (OR=0.915, 95%CI:0.856-0.977, p=0.008). The presence of arthritis showed a trend toward association (OR=5.191, 95%CI:0.960-28.065, p=0.056) but did not reach statistical significance. No significant differences were observed in gender, ANCA subtypes, major organ involvement, or comorbidity burden between groups.</p><p><strong>Conclusion: </strong>Our findings suggest that younger age is associated with a higher likelihood of cyclophosphamide resistance in GPA, potentially indicating a more aggressive disease course. Although arthritis showed a potential association with resistance, further studies are needed to confirm its role.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"37 1","pages":"199-205"},"PeriodicalIF":2.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147843671","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}
引用次数: 0
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