Rheumatology International最新文献

{"title":"Defining muscle-dominant ANCA-associated vasculitis: the importance of systematic muscle MRI assessment.","authors":"Tahir Saygın Öğüt, Mert Ünal, Veli Yazisiz","doi":"10.1007/s00296-026-06277-z","DOIUrl":"https://doi.org/10.1007/s00296-026-06277-z","url":null,"abstract":"","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892313","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}

{"title":"Handgrip strength relative to age- and sex-specific reference values in hospitalised patients with highly active rheumatoid arthritis: a single-centre cross-sectional study.","authors":"Umida Khojakulova, Marlen Yessirkepov, Bekaidar Nurmashev, Olena Zimba, Maidan Mukhamediyarov","doi":"10.1007/s00296-026-06292-0","DOIUrl":"https://doi.org/10.1007/s00296-026-06292-0","url":null,"abstract":"<p><p>Handgrip strength (HGS) is a valuable indicator of muscle function that can be used to identify patients with rheumatoid arthritis (RA) at risk of sarcopenia and other functional impairments. The aim of this study was to describe grip strength (HGS) in patients with rheumatoid arthritis (RA), to assess the frequency of low HGS, to quantify grip strength relative to age- and sex-specific reference values, and to identify the clinical factors associated with HGS. In this single-centre cross-sectional study, 70 patients with RA had their HGS measured sequentially on both arms using an electronic hand dynamometer, with three measurements taken per arm; the higher of the HGSmeasurementse was taken as the maximum. Low HGS was defined as < 27 kg for men and < 16 kg for women. The maximum HGS was additionally expressed as an age- and sex-adjusted z-score relative to normative data; deviations from the norm were tested using the Wilcoxon signed-rank test (with an assumed median z = 0). Spearman's correlation and multivariate linear regression were used to determine the presence of an association with the maximum HGS value. The patients were predominantly women (n = 55; 78.6%), with a mean age of 53.6 ± 13.3 years, presenting with a long-standing course of RF-positive, erosive, highly active disease (median DAS28-ESR 5.6). The mean maximum HGS was 23.9 ± 8.8 kg. Low HGS was observed in 28.6% of patients (20% of women, 60% of men). Compared with age- and sex-matched norms, grip strength was significantly lower in men (median z = -1.71, p = 0.001) and in women (median z = -0.55, p < 0.001) . HGS scores were inversely correlated with age (ρ = -0.4, p = 0.001) and current smoking (ρ = -0.41, p = 0.001) and positively associated with body mass index (BMI) (ρ = 0.35, p = 0.003). Older age was the only variable independently associated with lower grip strength (B = -0.21 kg/year, p = 0.012). In this study, grip strength was low relative to established sarcopenia thresholds and age- and sex-specific reference values.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892324","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}

{"title":"Lack of association between the Dudley Inflammatory Bowel Symptom Questionnaire and fecal calprotectin in axial spondyloarthritis: a cross-sectional study.","authors":"Meryem Ozoglu, Neslihan Gokcen, Duygu Temiz Karadag, Ozlem Ozdemir Isik, Ayse Cefle, Ayten Yazici","doi":"10.1007/s00296-026-06290-2","DOIUrl":"https://doi.org/10.1007/s00296-026-06290-2","url":null,"abstract":"<p><p>To investigate the relationship between gastrointestinal symptom burden assessed by the Dudley Inflammatory Bowel Symptom Questionnaire (DISQ), fecal calprotectin (FC), a non-invasive biomarker suggestive of intestinal inflammation, disease activity, and clinical characteristics in patients with axial spondyloarthritis (axSpA). In this cross-sectional study, 174 patients with axSpA were enrolled. Gastrointestinal symptom burden was assessed using the DISQ, disease activity using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), functional status using the Bath Ankylosing Spondylitis Functional Index (BASFI), and health-related quality of life using the Short Form-36 (SF-36). FC levels were measured by enzyme-linked immunosorbent assay. Correlation analyses, receiver operating characteristic (ROC) analyses, and multivariable logistic regression models were performed. FC levels were normal (< 50 μg/g) in 52.9%, mildly elevated (50-200 μg/g) in 38.5%, and markedly elevated (> 200 μg/g) in 8.6%. DISQ scores demonstrated a moderate positive correlation with BASDAI (rs = 0.432, p < 0.001) and a weak positive correlation with BASFI (rs = 0.248, p < 0.001), while showing moderate inverse correlations with all SF-36 domains (rs = - 0.310 to -0.470, all p < 0.001). In contrast, DISQ scores were not associated with FC levels (rs = 0.066, p = 0.386), ESR (rs = 0.013, p = 0.868), or CRP (rs = 0.029, p = 0.706). ROC analyses demonstrated poor discriminatory performance between DISQ and FC (AUC 0.529 and 0.475, respectively). In multivariable analyses, BASDAI independently predicted clinically significant gastrointestinal symptoms (OR 1.635, 95% CI 1.285-2.081) and elevated FC levels (OR 1.254, 95% CI 1.004-1.567), whereas neither DISQ nor FC independently predicted the other. Gastrointestinal symptom burden assessed by the DISQ was associated with disease activity, functional impairment, and reduced quality of life, but not with FC levels in axSpA.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892252","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}

{"title":"Evaluation of echocardiographic features and cardiac biomarkers in patients with AA amyloidosis: a retrospective comparative study with patients with al amyloidosis and hypertrophic cardiomyopathy.","authors":"Murat Bektaş, Mehmet Güven Günver, Ömer Uludağ, Ezgi Şahin, Besim Fazıl Ağargün, Gizem Dağcı, Nevzat Koca, Emin Oğuz, Shirkhan Amikishiyev, Burak İnce, Metban Güzel Mastanzade, Simge Erdem, Mehmet Aydoğan, Yasemin Yalçınkaya, Bahar Artım-Esen, Murat İnanç, Pelin Özer Karaca, Sevgi Kalayoğlu Beşışık, Ahmet Gül","doi":"10.1007/s00296-026-06289-9","DOIUrl":"https://doi.org/10.1007/s00296-026-06289-9","url":null,"abstract":"<p><p>Cardiac amyloidosis (CA) is a well-known feature of AL amyloidosis (AL-A) and transthyretin amyloidosis, and its detection is important due to its association with a poor prognosis. In this study, we aimed to evaluate cardiac dysfunction with echocardiographic features alongside cardiac biomarkers in patients with AA-A in a controlled design with appropriate control groups. This retrospective observational study was conducted at a referral center for amyloidosis in Istanbul, Türkiye. The study population consisted of patients with AA-A, as well as patients with AL-A and hypertrophic cardiomyopathy (HCM) as diseased controls. We included 139 patients (56% male) with AA-A, 89 patients (52% male) with AL-A, and 54 (57.4% male) patients with HCM in the study. The mean interventricular septum diameter (IVSd) was 11.8 ± 2.3, 14.6 ± 3.7, and 17.4 ± 4.3 mm in AA-A, AL-A, and HCM, respectively (p < 0.001 for each). The mean posterior wall diameter (PWd) was 10.5 ± 1.7, 12.9 ± 2.8, and 12 ± 1.6 mm in AA-A, AL-A, and HCM, respectively (p < 0.001 for AA-A and AL-A and AA-A and HCM; p = 0.056 for AL-A and HCM). The fulfillment of the Gertz et al. criteria was 34.5% in AA-A, 68.5% in AL-A, and 0% in HCM (p < 0.001 for each). Additionally, 4.3% in AA-A, 27.3% in AL-A, and 2% in the HCM group fulfilled the PWd > 12.5 mm plus troponin > 13.15 pg/mL plus serum creatinine (Screa)>1.085 mg/dL criterion. In multivariate analysis, PWd and left ventricular diastolic dysfunction were associated with suspected CA according to the Gertz et al. criteria in patients with AA-A. In Cox regression analysis, higher PWd and reduced EF were associated with increased mortality in patients with AA-A. Survival analysis revealed lower survival in patients who met the Gertz et al. criteria, the Gertz et al. plus granular echogenicity, the PWd > 12.5 mm plus troponin > 13.15 pg/mL plus Screa>1.085 mg/dL criteria, and patients who had increased PWd, reduced ejection fraction, advanced diastolic dysfunction, and granular echogenicity. In ROC analysis, troponin > 24.5 pg/mL had 100% sensitivity and 66.1% specificity, and pro-BNP > 636.5 pg/mL had 94.4% sensitivity and 53.7% specificity, yielding higher mortality in patients with AA-A. A considerable proportion of AA-A patients may be at risk of developing cardiac dysfunction that may be suggestive of CA. Increased posterior wall thickness, granular echogenicity, and advanced diastolic dysfunction may be suggestive echocardiographic features of suspected CA in patients with AA-A. These findings of myocardial dysfunction warrant further investigation to evaluate cardiac involvement in AA-A patients. Prospective studies with histopathological and/or MRI confirmation are needed to better define the causes of cardiac dysfunction in AA-A.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892273","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}

{"title":"AA amyloidosis in familial Mediterranean fever: a comparative study of clinical and genetic features between Algerian probands and their relatives.","authors":"G Khellaf, L Kaci, M R Bahriz, D Ait-Idir, H Boucenna, L Debchi, H Rafa-Debbah, Y Rahou, S Missoum, S Chelghoum, M Benabadji, A Benziane","doi":"10.1007/s00296-026-06283-1","DOIUrl":"https://doi.org/10.1007/s00296-026-06283-1","url":null,"abstract":"<p><p>AA amyloidosis is the most severe complication of familial Mediterranean fever (FMF). Why some patients develop amyloidosis while genotype-identical relatives remain unaffected is unknown. We assessed whether genetic counseling helps to identify at-risk relatives and whether inflammatory activity, rather than MEFV genotype, drives amyloidosis. We conducted a single-center comparative study (1998-2025) including 52 Algerian FMF probands with biopsy-proven AA amyloidosis and 30 first- or second-degree relatives with biallelic MEFV mutations who also had FMF but no amyloidosis. All underwent clinical, laboratory and MEFV genotyping (exons 2,3,5,10). Only individuals with two MEFV mutations (homozygous or compound heterozygous) were included. Multivariable logistic regression identified independent predictors of amyloidosis. The M694I/M694I genotype was equally frequent in both groups (71.2% vs. 66.7%, p = 0.67). In univariable analysis, it was not associated with amyloidosis (OR 1.23, 95% CI 0.48-3.16, p = 0.67). Independent drivers of amyloidosis were identified by multivariable logistic regression: attack duration > 72 h (adjusted odds ratio [aOR] 3.21, 95% CI 1.41-7.31, p = 0.006), attack interval < 3 months (aOR 4.12, 95% CI 1.77-9.58, p < 0.001), and baseline CRP > 100 mg/L (aOR 2.91, 95% CI 1.23-6.88, p = 0.014). Only 9.6% of amyloidosis patients achieved normalised CRP (< 5 mg/L) under colchicine, compared to 86.7% of relatives (p < 0.001). Strikingly, among amyloidosis patients who achieved CRP normalisation, 57.7% required 1.5 mg/day of colchicine and 7.7% required 2 mg/day, whereas 86.7% of amyloid-free relatives achieved normalisation with only 1 mg/day, highlighting the higher inflammatory burden in patients who develop amyloidosis despite carrying the same MEFV genotype. Genetic counseling allowed screening of 30 relatives without amyloidosis. Inflammation, not MEFV genotype, dictates AA amyloidosis in FMF. Genetic counseling is essential to identify at-risk relatives. Treatment should target complete CRP normalisation regardless of genotype.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892286","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}

{"title":"Suspected shrinking lung syndrome: a rare pulmonary manifestation of systemic lupus erythematosus-a case-based review of an underrecognized condition.","authors":"Maciej Maj, Maria Oknińska, Joanna Robaczyńska, Martyna Dziewit, Marzena Olesińska, Maria Maślińska","doi":"10.1007/s00296-026-06291-1","DOIUrl":"10.1007/s00296-026-06291-1","url":null,"abstract":"<p><p>Shrinking lung syndrome (SLS) is a rare pulmonary manifestation of systemic lupus erythematosus (SLE). It is characterized by unexplained dyspnea, restrictive ventilatory defect, and diaphragmatic elevation without interstitial lung disease (ILD). A 32-year-old man with SLE presented with progressive dyspnea initially attributed to pleural and infectious processes. As systemic features evolved, SLE was confirmed. Pulmonary function tests (PFTs) showed severe restriction (forced vital capacity-FVC-28%, total lung capacity-TLC-37%), with elevated hemidiaphragm and no alternative pulmonary pathology, supporting the diagnosis of SLS. Treatment with highdose glucocorticoids, rituximab, and mycophenolate mofetil resulted in marked clinical improvement, substantial recovery of lung function (FVC 65%), and successful glucocorticoid withdrawal. A structured literature review (January 2021-March 2026) identified 19 studies reporting 37 SLS cases. Dyspnea was the predominant manifestation, frequently accompanied by pleuritic chest pain and dry cough. Restrictive ventilatory defects were consistently reported, with significantly reduced FVC, TLC, and transfer factor of the lung for carbon monoxide values. Imaging most commonly demonstrated elevated hemidiaphragm(s) and reduced lung volumes without ILD. Treatment predominantly involved glucocorticoids, often combined with steroid-sparing agents. Rituximab was frequently used as a steroid-sparing agent in severe/refractory cases and was generally associated with clinical and functional improvement. Based on the presented case and cases identified in literature, SLS remains a diagnostic challenge and should be considered in patients with SLE presenting with unexplained dyspnea and restrictive physiology. Early recognition and prompt immunosuppressive therapy may improve outcomes.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13525941/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148857756","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":"Mental- and physical-health comorbidity are strongly associated in idiopathic inflammatory myopathies: a multinational cross-sectional e-survey.","authors":"Samuel Katsuyuki Shinjo, Lekshmi Minikumari Rahulan, Meera Shah, Anchit Chauhan, Suhana Hussain, Paula Jordan, Meghna Lama, Oladipo Kunle Afolayan, Vincenzo Venerito, Tamar B Rubinstein, Ioannis Parodis, Elena Nikiphorou, Joanna Makowska, Aleksandra Opinc-Rosiak, Rada Miskovic, Marcin Milchert, Dimitri Luz Felipe da Silva, Vahed Maroufy, Vikas Agarwal, Latika Gupta","doi":"10.1007/s00296-026-06262-6","DOIUrl":"10.1007/s00296-026-06262-6","url":null,"abstract":"<p><p>Comorbidities are associated with morbidity, function, and survival in idiopathic inflammatory myopathies (IIM), but subtype-specific patterns, mental-physical comorbidity relationships, and patient-reported outcomes remain incompletely characterized. We hypothesized that multimorbidity in IIM is heterogeneous, associated with demographic, clinical, and psychosocial factors, and linked to differences in physical function and fatigue. We studied 1028 adults with self-reported, specialist-confirmed IIM. Comorbidities were assessed using the Functional Comorbidity Index and categorized into basic, complex, mental-health, and autoimmune domains. Logistic regression identified factors associated with mental- and physical-health comorbidity; clustering defined comorbidity patterns. Participants were predominantly female (71.3%), median age 59.0 years. Overall, 46.7% had basic multimorbidity, 15.4% complex multimorbidity, 33.6% mental-health comorbidity, and 14.7% autoimmune multimorbidity. Interstitial lung disease was concentrated in anti-synthetase syndrome (50.7%) and polymyositis (42.6%), while hypertension (37.8%) and malignancy (35.0%) were concentrated in inclusion body myositis. Chronic pain/fatigue syndromes were associated with mental-health burden (63.2% vs. 27.5%, p < 0.001). Mental-health comorbidity was associated with younger age (OR 0.98/year), complex multimorbidity (OR 2.85), immunosuppressive therapy (OR 1.53), and lower family support (OR 0.94/unit). Physical-health comorbidity was independently associated with older age (OR 1.05/year), mental-health comorbidity (OR 3.96), and overlap myositis (OR 3.33). Clustering identified three multimorbidity profiles: low-burden (49.3%), cardiometabolic/malignancy-predominant with low mental-health burden (22.9%), and pain-fatigue/mental-health-predominant (27.7%), which differed significantly in physical function and fatigue. Multimorbidity in IIM is common, heterogeneous, and shows a strong concurrent relationship between mental- and physical-health comorbidity, supporting integrated mental-health screening and subtype-tailored surveillance.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13526107/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148857697","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 characteristics, organ damage and survival in idiopathic inflammatory myopathies: a long-term retrospective cohort study.","authors":"Ezgi Sahin, Omer Uludag, Yasemin Yalcinkaya, Bahar Artim-Esen, Ahmet Gul, Murat Inanc","doi":"10.1007/s00296-026-06288-w","DOIUrl":"https://doi.org/10.1007/s00296-026-06288-w","url":null,"abstract":"<p><p>To compare the clinical phenotype, organ damage, and survival of patients with antisynthetase syndrome (ASyS) and other idiopathic inflammatory myopathies (OIIM) in a long-term, single-center retrospective cohort. We retrospectively analyzed patients with idiopathic inflammatory myopathy who fulfilled the Bohan and Peter criteria, the EULAR/ACR classification criteria and had available myositis specific autoantibody results. This cohort comprises patients followed in our autoimmune connective tissue disease clinic between 1991-2024. Demographic, clinical, laboratory, autoantibody, organ damage, and survival data were compared between ASyS and OIIM groups. Myositis Damage Index was used for assessing organ damage at the sixth month of follow-up and the last visit. Of the 122 patients, 35 (28.7%) were classified as ASyS (85.7% female) and 87 as OIIM (59.8% female). Median age at diagnosis was 47 and 46 years, respectively. The mean follow-up duration of the overall cohort was 84.48 ± 77.95 months. Among patients with ASyS, anti-Jo-1 was the most frequent autoantibody detected (88.6%), followed by anti-PL-7 in 8.6% and anti-KS in 2.8%. Mechanic's hands, arthritis, and interstitial lung disease were more frequent in the ASyS group. Dysphagia was more frequent in the OIIM group (ASyS: 8.6%; OIIM: 37.9%, p = 0.003). Anti-Ro-52 positivity was more common in ASyS than in OIIM (ASyS: 42.9%, OIIM: 21.8%, p = 0.034). C-reactive protein, leukocyte, and neutrophil levels were higher in the ASyS group at baseline. The median Myositis Damage Index score at the sixth month was nominally higher in ASyS than in OIIM (median MDI: 3 vs. 2, p = 0.046). However, MDI scores were comparable between the two groups at the last assessment. In autoantibody-based subgroup analyses, higher early damage scores were observed in ASyS than in anti-TIF1γ-positive patients (median MDI = 3 vs 1.5, p = 0.033). Interstitial lung disease (ILD) was more frequently present in ASyS, whereas malignancy was more frequent among anti-TIF1γ-positive patients. The mortality rate was 14.3% in the ASyS group and 14.9% in the OIIM group. Survival analysis did not reveal a statistically significant difference at the end of the follow-up between ASyS and OIIM groups. In this long-term retrospective cohort, patients with ASyS showed a distinct clinical phenotype compared with OIIM. Sixth-month MDI scores were nominally higher in ASyS and may partly reflect the higher burden of ILD; however, MDI subdomain analyses were not available. No statistically significant difference in survival was detected between groups, although this finding should be interpreted cautiously because of the small number of deaths. Overall, these findings should be considered exploratory and hypothesis-generating and require confirmation in larger multicenter prospective cohorts.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148857713","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}

{"title":"From ANCA-mediated vascular injury to coronary microvascular and myocardial involvement in granulomatosis with polyangiitis: an immunocardiology perspective.","authors":"Mateusz Lucki, Bogna Grygiel-Górniak, Ewa Lucka, Sylwia Iwańczyk, Maciej Lesiak","doi":"10.1007/s00296-026-06282-2","DOIUrl":"10.1007/s00296-026-06282-2","url":null,"abstract":"<p><p>Granulomatosis with polyangiitis (GPA) is a prototypical antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by necrotizing inflammation of small- and medium-sized vessels with granulomatous features. Although advances in immunosuppressive therapy have markedly improved short-term survival, long-term outcomes remain limited by cumulative organ damage, treatment-related toxicity, and an excess burden of cardiovascular morbidity and mortality. Cardiovascular risk in GPA appears to reflect a combination of conventional risk factors, treatment-related metabolic effects, renal dysfunction, systemic inflammation, and disease-associated vascular injury and may therefore extend beyond accelerated epicardial atherosclerosis alone. Cardiac magnetic resonance (CMR) studies in GPA and the broader AAV population have identified myocardial abnormalities, including late gadolinium enhancement, inflammatory changes, and fibrotic remodeling, sometimes in patients without overt cardiac symptoms. However, these observations do not establish the prevalence, mechanisms, or prognostic significance of coronary microvascular dysfunction (CMD) specifically in GPA. Neutrophil-mediated inflammation, complement amplification, endothelial dysfunction, oxidative stress, and thromboinflammatory pathways are established components of AAV pathophysiology. Their potential contribution to coronary microvascular dysfunction, impaired myocardial perfusion, and subsequent myocardial remodeling in GPA is biologically plausible but remains insufficiently demonstrated in dedicated human studies. Accordingly, the proposed links between AAV-related vascular inflammation, repetitive low-grade ischemia, fibrosis, and arrhythmogenesis should currently be considered hypothesis-generating and partly extrapolated from experimental AAV models and the broader cardiovascular literature. Unlike previous publications addressing cardiovascular manifestations across the wider AAV spectrum, the present review adopts a GPA-specific and coronary microcirculation-centered perspective. It integrates available immunopathological and imaging evidence with contemporary CMD and ischemia with non-obstructive coronary arteries frameworks while distinguishing established observations from proposed mechanistic relationships. This review also discusses the potential roles of advanced cardiovascular imaging, individualized risk assessment, and targeted cardiovascular evaluation within an emerging immunocardiology framework. Prospective GPA-specific studies are needed to determine the clinical significance of CMD and establish whether targeted cardiovascular monitoring and cardioprotective strategies improve patient outcomes.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13518386/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148831977","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":"Ankylosing spondylitis following chronic brucellosis: a causal association or separate pathology? A case-based review.","authors":"Oleksandr Boichuk, Oleksandra Pryshliak, Andrii Protsyk, Zoriana Tylishchak, Bohdana Doskaliuk","doi":"10.1007/s00296-026-06285-z","DOIUrl":"10.1007/s00296-026-06285-z","url":null,"abstract":"<p><p>Brucellosis is a widespread zoonotic infection that often follows a chronic course and may involve the osteoarticular system. Patients with brucellosis-related musculoskeletal manifestations are frequently evaluated by rheumatologists and may be misdiagnosed with rheumatic diseases, including ankylosing spondylitis. We present the case of a 45-year-old female patient who was diagnosed with osteoarticular brucellosis approximately 20 years ago and was successfully treated with etiotropic therapy. Eighteen years later, she developed back pain that worsened at night and did not improve with rest, accompanied by morning stiffness. Further evaluation revealed negative specific anti-Brucella antibodies, HLA-B27 positivity confirmed by polymerase chain reaction, and magnetic resonance imaging findings supporting the diagnosis of ankylosing spondylitis/axial spondyloarthritis. A literature review including 21 relevant sources was conducted to analyse the diagnostic overlap and possible coexistence of brucellosis and ankylosing spondylitis. The reviewed evidence, together with the clinical course of the present patient, supports the interpretation that ankylosing spondylitis developed as a separate pathological condition rather than as a direct consequence of previously treated brucellosis. This case highlights the importance of careful differential diagnosis between brucellosis-related osteoarticular involvement and axial spondyloarthritis, particularly in patients from brucellosis-endemic regions or with relevant epidemiological exposure.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"46 9","pages":""},"PeriodicalIF":3.5,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819491","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}