Amaranta Manrique de Lara, Tirsa Colmenares-Roa, Laura Athié, Yurilis Fuentes-Silva, Cinthya Cadena-Trejo, María Fernanda Ramírez-Flores, Alfonso Gastelum-Strozzi, Rosana Quintana, Bernardo A Pons-Estel, Ingris Peláez-Ballestas
{"title":"Barriers and facilitators associated with diagnostic and treatment delays in lupus in the Global South and North: a systematic review of qualitative and mixed methods studies.","authors":"Amaranta Manrique de Lara, Tirsa Colmenares-Roa, Laura Athié, Yurilis Fuentes-Silva, Cinthya Cadena-Trejo, María Fernanda Ramírez-Flores, Alfonso Gastelum-Strozzi, Rosana Quintana, Bernardo A Pons-Estel, Ingris Peláez-Ballestas","doi":"10.1007/s00296-025-05875-7","DOIUrl":"https://doi.org/10.1007/s00296-025-05875-7","url":null,"abstract":"<p><p>Currently, there is no specific definition of delay in the diagnosis and treatment of systemic lupus erythematosus. This study aimed to describe the experiences of diagnostic and treatment delays in lupus, along with their associated barriers and facilitators, and to contrast this phenomenon in the Global South and North. A qualitative systematic review of the literature was conducted following the Joanna Briggs Institute and PRISMA guidelines. A targeted electronic search strategy was performed in Scopus, PubMed, and Web of Science. This protocol was registered with the international prospective register of systematic reviews (PROSPERO CRD42023456508). From 1,135 identified registries, 12 (ten qualitative and two mixed methods) articles were included in the analysis, most from the Global North. None of the publications clearly define diagnostic delay in lupus. Patients, caregivers, and healthcare professionals identified a range of barriers and facilitators. Misdiagnosis, miscommunication, the lack of rheumatology knowledge and education, and the diagnostic pilgrimage were barriers reported in both regions. In the Global South, barriers were related to a fragmented and complex healthcare system, stigma, and economic and geographic difficulties. In the Global North, physician-patient interactions and reliance on antinuclear antibody testing were significant barriers. The reported facilitators mostly comprised individual-level factors like self-care and resilience. This review highlights the lack of a clear definition of diagnostic and treatment delays in lupus, integrating the perspectives of patients, family and caregivers, and healthcare providers. The contrasting emphasis on external context factors in the Global South may reflect underlying health inequalities.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"121"},"PeriodicalIF":3.2,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jonas B Thorlund, Eivind S Skarpsno, Jonas J Vestergaard, Søren T Skou, Dorte T Grønne, Ewa M Roos, Henrik B Vaegter
{"title":"Sleep problems and insomnia are common and associated with pain intensity, number of comorbidities and analgesic use in patients with knee and hip osteoarthritis: a cross-sectional study using data from the good life with osteoarthritis in Denmark (GLA:D<sup>®</sup>) registry.","authors":"Jonas B Thorlund, Eivind S Skarpsno, Jonas J Vestergaard, Søren T Skou, Dorte T Grønne, Ewa M Roos, Henrik B Vaegter","doi":"10.1007/s00296-025-05878-4","DOIUrl":"https://doi.org/10.1007/s00296-025-05878-4","url":null,"abstract":"<p><p>To assess the difference in prevalence of sleep problems and insomnia in patients with knee or hip osteoarthritis (OA), and explore characteristics associated with sleep problems and insomnia. We included 8,162 knee/hip OA patients enrolled in supervised exercise and patient education through the Good Life with osteoArthritis in Denmark (GLA:D<sup>®</sup>) program. We assessed presence of sleep problems (yes/no), followed by the Insomnia Severity Index 3-item (ISI-3) questionnaire among those with sleep problems (Insomnia: ISI-3 score ≥ 7). Characteristics associated with sleep problems/insomnia was estimated for knee and hip OA patients separately (prevalence ratios [PR]). In total, 68% (n = 3,539) and 64% (n = 1,807) of knee and hip OA patients reported sleep problems, respectively, corresponding to a PR of 1.06 (95% CI 1.03 to 1.10). Prevalence of insomnia was 17% (n = 943) and 20% (n = 528) for those with knee and hip OA, respectively (PR 1.18 [95% CI 1.07 to 1.30]). Large overlap between characteristics associated with sleep problems and insomnia were observed. Characteristics most strongly associated with higher prevalence of insomnia were pain intensity ≥40 mm VAS (knee: PR 2.39 [95% CI 2.08 to 2.74]; hip: PR 2.54 [95% CI 2.10 to 3.07], a high number of comorbidities, and analgesic use in both patients with knee and hip OA. Sleep problems and insomnia are highly prevalent among primary care patients with knee and hip OA, and slightly more common in hip OA patients. Prevalence of insomnia was substantially higher among patients with more comorbidities, higher pain intensity and analgesic use.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"123"},"PeriodicalIF":3.2,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12031806/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Anterior segment alterations in fibromyalgia syndrome: a cross-sectional study on dry eye disease, meibomian gland dysfunction, and astigmatism.","authors":"Duygu Topaktaş Emekli, Eda Sahutoglu, Gülşah Yaşa Öztürk, Burhan Fatih Kocyigit","doi":"10.1007/s00296-025-05872-w","DOIUrl":"https://doi.org/10.1007/s00296-025-05872-w","url":null,"abstract":"<p><p>Fibromyalgia syndrome (FMS) is an ongoing disorder distinguished by its multifaceted characteristics. There has been increasing interest in its ophthalmologic aspects. This study evaluated the anterior segment parameters of the eye in patients with FMS, comparing them to healthy controls. Furthermore, correlations with disease activity were analyzed. This research was structured as a cross-sectional and comparative analysis. Data was collected at a tertiary care education and research hospital from August 20, 2024, to November 15, 2024. The participants' central corneal thickness, uncorrected and best-corrected visual acuities, refraction parameters, intraocular pressure, meibomian gland dysfunction (MGD), and dry eye disease (DED) parameters were evaluated. There were no significant differences in visual acuity, spherical equivalent, intraocular pressure, and central corneal thickness between FMS and healthy controls (p > 0.05). The median astigmatism in the right eye (diopter) was - 0.50 (-8.25-0) for the FMS group and - 0.12 (-1.75-0.50) for the control group (p = 0.012). The left eye data were - 0.50 (-2-1.50) for the FMS group and 0 (-1.75-0.75) for the control group (p = 0.056). MGD was identified in 26 (36.6%) individuals in the FMS group, whereas it was identified in 3 (4.3%) participants in the control group (p < 0.001). DED was detected in 43 (60.6%) participants in the FMS group and 15 (21.4%) participants in the control group (p < 0.001). FMS patients had higher rates of astigmatism, MGD, and DED compared to healthy controls, underscoring the necessity for routine anterior segment assessments. These data suggest possible ocular involvement in FMS, which warrants further research.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"120"},"PeriodicalIF":3.2,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12031864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143978075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical features and diagnostic challenges in crowned dens syndrome: a systematic review and meta-analysis.","authors":"Omar Hamdan, Yazeed Alajlouni, Qais Mussa, Ahmad Alsawalmeh, Qutaiba Sabbah, Muna Al-Zou'bi, Asem Abuhamdan, Fatima Alnaimat","doi":"10.1007/s00296-025-05884-6","DOIUrl":"https://doi.org/10.1007/s00296-025-05884-6","url":null,"abstract":"<p><p>Crowned Dens Syndrome (CDS) is an acute inflammatory neck pain linked to Calcium Pyrophosphate crystal deposition surrounding the odontoid process. This systematic review and meta-analysis aim to illuminate the Features and Diagnostic Challenges of CDS. PubMed, Web of Sciences, and Scopus databases were searched in July 2024 to include all studies on CDS without publication year or language restrictions. Demographic, clinical, laboratory, and radiological data, in addition to treatment regimens and outcomes, were extracted and analyzed. PROSPERO registration number is CRD42024575491. We analyzed 153 reports, including 196 CDS patients, with a mean age of 73.13 ± 12.95 years and an almost equal female-to-male ratio. Neck pain was the most common symptom (96.8%). CRP and ESR were elevated in 91.4% and 80.7% of cases, respectively. CT scans had the highest detection rate for abnormalities (97.3%), compared to MRI (68.4%) and plain radiographs (79.4%). NSAIDs were the most frequently used treatment (62.5%), with improvement observed in 96.6% of cases. Meningitis was the most common misdiagnosis (21.4%), leading to a higher likelihood of plain radiographs and lumbar punctures. In contrast, patients with neck tenderness and restricted range of motion had a lower risk of misdiagnosis. Patients who were initially misdiagnosed were more likely to undergo LP (13.1% Vs. 34.8%; P-value < 0.001). CDS is an elderly disease that usually presents as acute neck pain with elevated inflammatory markers and is frequently misdiagnosed as meningitis. Recognizing key clinical features may improve diagnosis and management.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"124"},"PeriodicalIF":3.2,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Oğuz Konal, Furkan Bölen, Tolga Sinan Güvenç, Özlem Pehlivan, Sevilay Batıbay, Fatma Betül Özcan, Ayşe Paralı, Feyza Aksu, Şeref Kul, Mustafa Çalışkan
{"title":"Gout and rheumatoid arthritis are associated with subclinical vascular damage, reduced brachial vasoreactivity and coronary microvascular dysfunction: a case-control study.","authors":"Oğuz Konal, Furkan Bölen, Tolga Sinan Güvenç, Özlem Pehlivan, Sevilay Batıbay, Fatma Betül Özcan, Ayşe Paralı, Feyza Aksu, Şeref Kul, Mustafa Çalışkan","doi":"10.1007/s00296-025-05868-6","DOIUrl":"https://doi.org/10.1007/s00296-025-05868-6","url":null,"abstract":"<p><p>Gout and rheumatoid arthritis (RA) are distinct disorders, but both of these inflammatory arthropaties are associated with a relatively similar risk of atherosclerotic cardiovascular disease. We aimed to assess carotid intima-media thickness (CIMT), flow-mediated vasodilatation (FMD) and coronary flow reserve (CFR) in both disorders to understand and compare the extent of vascular damage. Patients with recently diagnosed gout (n = 30) and RA (n = 40) without previous cardiovascular disease were included to the present study, and 45 healthy volunteers served as controls. All participants underwent ultrasonographic examinations to measure CIMT, FMD and CFR. In unadjusted analysis, both gout and RA were associated with a significantly higher CIMT and significantly lower CFR as compared to controls, while FMD was only lower in the gout group. After adjustment, there was a significantly higher odds ratio (OR) for CIMT (OR:7.02, 95%CI:2.45-20.58 for gout and OR:2.86, 95%CI:1.27-6.57 for RA) and a lower OR for CFR (OR:0.21, 95%CI:0.08-0.55 for gout and OR:0.17, 95%CI:0.07-0.45 for RA) in both groups, while only gout patients had a lower OR for FMD (OR:0.21, 95%CI:0.08-0.55). There were no significant differences or evidence of a difference between gout and RA patients for CIMT, FMD or CFR. Gout and RA are associated with a similar degree of vascular damage, which may explain the similar incidence of atherosclerotic cardiovascular disease in both diseases.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"117"},"PeriodicalIF":3.2,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12018609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Si Chen, Rui Nie, Haixia Luan, Xiaoran Shen, Yan Wang, Yuan Gui, Xiaoli Zeng, Hui Yuan
{"title":"Association of acute kidney injury with 1-year mortality in granulomatosis with polyangiitis patients: a cohort study using mediation analyses and machine learning.","authors":"Si Chen, Rui Nie, Haixia Luan, Xiaoran Shen, Yan Wang, Yuan Gui, Xiaoli Zeng, Hui Yuan","doi":"10.1007/s00296-025-05822-6","DOIUrl":"https://doi.org/10.1007/s00296-025-05822-6","url":null,"abstract":"<p><p>To investigate the correlation between acute kidney injury (AKI) and 1-year mortality in patients with granulomatosis with polyangiitis (GPA). Clinical data for GPA patients were extracted from the MIMIC-IV (version 3.0) database. Logistic and Cox regression analyses, Kaplan-Meier (KM) survival analysis, and mediation effect analysis were used to assess the association between AKI, renal function indicators, and 1-year mortality in GPA patients. Predictive models were constructed using machine learning algorithms, and tree-based feature selection was applied to evaluate the contributions of AKI and renal function indicators to mortality prediction. A total of 127 GPA patients were included in the analysis. Multivariate logistic regression identified AKI (OR > 1, P < 0.05) as a significant predictor of 1-year mortality. Similarly, multivariate Cox regression analysis revealed AKI (HR > 1, P < 0.05) as an independent risk factor for 1-year mortality. KM survival analysis demonstrated that GPA patients with AKI had significantly lower survival rates than those without AKI (P < 0.0001). Additionally, renal function indicators modestly mediated the relationship between AKI and 1-year mortality in GPA patients. The machine learning analysis indicated that the random forest algorithm performed the best, with an area under the curve of 0.894. Feature selection using tree model analysis highlighted both AKI and renal function indicators as significant contributors to mortality prediction in GPA patients. Our study suggested AKI was an independent risk factor for increased 1-year mortality in GPA patients. Additionally, renal function indicators partially mediated the relationship between AKI and 1-year mortality in these patients.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"118"},"PeriodicalIF":3.2,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144010120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hatice Betigul Meral, Aylin Rezvani, Sena Tolu, Ahmet Usen, Muhammed Furkan Dasdelen
{"title":"Structural changes in the upper trapezius muscle of fibromyalgia patients identified by quantitative ultrasonography: a cross-sectional study.","authors":"Hatice Betigul Meral, Aylin Rezvani, Sena Tolu, Ahmet Usen, Muhammed Furkan Dasdelen","doi":"10.1007/s00296-025-05871-x","DOIUrl":"https://doi.org/10.1007/s00296-025-05871-x","url":null,"abstract":"<p><p>The heterogeneity of symptoms among patients with fibromyalgia (FM) makes the development of standardized diagnostic criteria challenging. No imaging technique has reliably shown FM-related muscle changes to aid clinical assessment. This study aimed to quantitatively analyze the upper trapezius muscle in FM patients using B-mode ultrasonography and blob analysis and to examine its correlation with clinical parameters. A total of 34 female FM patients and 34 healthy controls were included in this cross-sectional study. B-mode ultrasonography was used to image the dominant-side upper trapezius muscle, and MATLAB-based blob analysis was performed to assess blob size, blob count, and echointensity. These measurements were correlated with disease severity indices, including the Central Sensitization Inventory (CSI), Visual Analog Scale (VAS) for pain, Fibromyalgia Impact Questionnaire (FIQ), 36-Item Short Form Survey (SF-36), and Beck Depression and Anxiety Inventories (BDI, BAI). FM patients had significantly higher total blob size (p < 0.001) and blob size per mm² (p < 0.001) than controls. Echointensity was significantly increased in the FM group (p = 0.009). Total blob size showed a moderate positive correlation with CSI scores (p = 0.002). Regression analysis indicated that pain-VAS was a significant predictor of total blob size per mm² (p < 0.001). Blob analysis demonstrated quantifiable muscle alterations in FM, supporting its potential role as an objective assessment tool. Given the correlation between muscle echotexture and FM severity, quantitative ultrasonography may contribute to a better understanding of FM pathophysiology.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"115"},"PeriodicalIF":3.2,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12014792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144045495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ali Kirik, Nilay Şahin, Merve Baykul, Hatice Bodur, Tuba Güler, Remzi Çevik, Sevcan Uğur, Yunus Durmaz, Ali Yavuz Karahan, Gül Devrimsel, Nuran Öz, Mehmet Nur Kaya, Yeşim Çağlar, Mehmet Tuncay Duruöz, Kemal Nas
{"title":"Low vaccination rates and awareness status in patients with rheumatoid arthritis: a nationwide cross-sectional survey study.","authors":"Ali Kirik, Nilay Şahin, Merve Baykul, Hatice Bodur, Tuba Güler, Remzi Çevik, Sevcan Uğur, Yunus Durmaz, Ali Yavuz Karahan, Gül Devrimsel, Nuran Öz, Mehmet Nur Kaya, Yeşim Çağlar, Mehmet Tuncay Duruöz, Kemal Nas","doi":"10.1007/s00296-025-05870-y","DOIUrl":"https://doi.org/10.1007/s00296-025-05870-y","url":null,"abstract":"<p><p>To examine the pneumococcal, haemophilus influenza, hepatitis A virus (HAV), and hepatitis B virus (HBV) vaccine rates and and predictors of vaccination among a Türkiye population of rheumatoid arthritis (RA) patients. In this multicenter, cross-sectional study, vaccination levels and related factors were questioned by face-to-face survey method during routine clinical examination of patients with RA followed in different regions of Türkiye. All statistical analyses were performed using the Statistical Package for the Social Sciences (SPSS) software package, version 26. A total of 715 patients (mean age 53.1 ± 13 years), of whom 552 (77.2%) were women, were included in this study. The rate of vaccine awareness education in the whole patient group was 40.1%. The vaccination rates for influenza, pneumococcal, HAV, and HBV were 34.3%, 21.8%, 12.3%, and 28.5%, respectively. It was noteworthy that the level of vaccine awareness education was a positive predictive factor affecting vaccination in all vaccine types (p < 0.001, for all vaccine types). The rates of low income were higher in the vaccinated group for pneumococcal (p < 0.001) and haemophilus influenza (p = 0.011) vaccine than in the unvaccinated group. The participants with living in a rural area were significantly higher in the unvaccinated group for HAV and HBV vaccine than in the vaccinated group (p < 0.001). The frequency of less than two medical visits per year was significantly higher in the unvaccinated group for pneumococcal (p < 0.014) and haemophilus influenza (p < 0.001 ) and HBV vaccine (p = 0.009) than in the vaccinated group. multivariate analysis found that rural area (for Haemophilus influenza and HAV vaccines) and financial income (Pneumococcal vaccine) in specifically were associated with increased vaccination rates (p < 0.001). Additionally, the rate of vaccination education was significantly higher in the vaccinated group for all vaccine types, which is noteworthy as a result of the positive effect of education on vaccination (p < 0.001). Vaccination awareness education and rates are low in primary prevention for RA patients. Vaccination awareness training is important in increasing the level of vaccine rates.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"116"},"PeriodicalIF":3.2,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12014830/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Argyro Konstantopoulou, Ioannis Tsoliakos, Maria M Berikopoulou, Aikaterini Kiorsavva, Maria Theochari, Panagiota Drosatou, Anna Messaritaki, Dimitra Dimopoulou
{"title":"A case-based review of IgA vasculitis complicated with gastrointestinal infections: insights from a norovirus-associated case in an adolescent.","authors":"Argyro Konstantopoulou, Ioannis Tsoliakos, Maria M Berikopoulou, Aikaterini Kiorsavva, Maria Theochari, Panagiota Drosatou, Anna Messaritaki, Dimitra Dimopoulou","doi":"10.1007/s00296-025-05876-6","DOIUrl":"https://doi.org/10.1007/s00296-025-05876-6","url":null,"abstract":"<p><p>Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a self-limited leukocytoclastic vasculitis targeting small-sized vessels. It is the most common vasculitis in children, but also targets adults who have usually worse prognosis. It typically presents with purpuric rash, arthritis, colicky abdominal pain and potential renal involvement. Gastrointestinal (GI) manifestations, although frequent, may be severe and occasionally complicated by infections. We present a 11-year-old male with a recent diagnosis of IgAV who was admitted with severe abdominal pain, hematemesis and hematochezia. Stool analysis identified Norovirus as the causative pathogen for the severe gastrointestinal symptoms. Treatment with proton pump inhibitors, high-dose corticosteroids and intravenous fluids resulted in symptoms resolution and clinical improvement. In addition, a case-based review of the literature was conducted to evaluate the prevalence of GI infections occurring after the development of IgAV that may result in severe complications or disease relapses. Thirty-five patients with a median age of 14 years were included in our study. Among these patients, 42.9% were diagnosed with a concomitant bacterial gastrointestinal pathogen, followed by 25.7% with viral gastroenteritis and 17.1% with parasitic gastrointestinal disease. Treatment was individualized, with 48.5% receiving corticosteroids and 8.6% receiving immunosuppressive therapy. This case and case-based review highlights the significance of careful management and monitoring of patients with IgAV complicated by infections, because severe complications can cause significant morbidity and mortality especially in adults.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"114"},"PeriodicalIF":3.2,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143994076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Koji Suzuki, Mitsuhiro Akiyama, Koichi Saito, Kanako Shimanuki, Yuko Kaneko
{"title":"Efficacy of tocilizumab monotherapy for autoimmune hemolytic anemia associated with idiopathic multicentric Castleman disease: a case-based review.","authors":"Koji Suzuki, Mitsuhiro Akiyama, Koichi Saito, Kanako Shimanuki, Yuko Kaneko","doi":"10.1007/s00296-025-05880-w","DOIUrl":"https://doi.org/10.1007/s00296-025-05880-w","url":null,"abstract":"<p><p>Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by generalized lymphadenopathy with distinctive histopathological features and systemic inflammation driven by excessive interleukin-6 (IL-6) production. Anemia due to chronic inflammation is a common manifestation of iMCD; however, this disease can also be complicated by autoimmune hemolytic anemia (AIHA). While tocilizumab (TCZ), an IL-6 receptor blockade, has demonstrated efficacy in treating iMCD, its therapeutic effect on AIHA secondary to iMCD remains unclear. Here, we report the first case of iMCD complicated by AIHA successfully treated with TCZ monotherapy, without the need for concurrent glucocorticoid therapy. Notably, AIHA improved along with the disappearance of autoantibodies under TCZ monotherapy, suggesting that IL-6 played a key role in the production of autoantibodies and causing AIHA secondary to iMCD. Furthermore, our literature review identified six other cases of iMCD with AIHA, five of which achieved favorable outcomes with a combination of TCZ and prednisolone, except for one case that developed anti-TCZ antibody. In two cases, AIHA improved without requiring an increase of prednisolone dose. These findings suggest that TCZ may represent a viable therapeutic option not only for iMCD itself but also for AIHA secondary to iMCD.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 5","pages":"113"},"PeriodicalIF":3.2,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143994088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}