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Introduction: Eosinophilic fasciitis (EF) is a rare inflammatory disorder marked by elevated eosinophils in tissues and blood. Patients typically present with symptoms such as skin swelling, pain, and induration, which can be mistaken for other connective tissue diseases. The pathogenesis of EF is not fully understood, though immune and autoimmune mechanisms appear to play significant roles. Diagnosis often involves biopsies, lab analyses, and imaging techniques. Current treatment mainly consists of corticosteroids, but further research is needed to assess various therapeutic strategies. Understanding EF is essential for improving patient outcomes and quality of life. Purpose: The purpose of this article is to enhance knowledge of eosinophilic fasciitis by examining its pathogenesis, immune mechanisms, and diagnostic challenges, as well as evaluating current diagnostic criteria and treatments. Given the rarity and complexity of EF, the study also seeks to clarify its differentiation from similar diseases and offer insights into effective therapies for early diagnosis and improved treatment outcomes. Conclusion: EF is a rare disease, and its complexity, due to diverse etiologies and variable treatment efficacy, poses significant challenges in clinical practice. In the near future, the publication of new cases and the initiation of clinical trials should contribute to the development of innovative therapeutic strategies and an effective treatment algorithm. Materials and methods: Our review is based on an analysis of material collected in 'Pubmed', 'Google Scholar' and other scientific articles using the keywords: Eosinophilic fasciitis, Shulman, fibrosis, hypergammaglobulinemia, eosinophilia. [ABSTRACT FROM AUTHOR]

Seronegative antiphospholipid syndrome (SN‐APS) is uncommon and challenging condition, which should be included in the differential diagnosis of stroke in young, since it can result in arterial thrombosis.SN‐APS is typically diagnosed by exclusion; however, it is crucial to recognize it in order to choose the best antithrombotic treatment to lower the recurrence rate. [ABSTRACT FROM AUTHOR]

Eosinophilic fasciitis (EF) is a rare autoimmune disorder affecting the connective tissue. It is characterized by painful inflammation and fibrosis, leading to sclerosis of fascia concerning limbs. Diagnosis is mostly based on clinical findings and confirmed by histopathological examination. Its rare occurrence and non-obvious course of this disease, mimicking more common syndromes, often causes diagnostic problems. During the diagnostic process, a number of diseases must be excluded: systemic scleroderma (SS), nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, or Churg-Strauss syndrome (EGPA). The following case report features a 67-year-old female and identifies the characteristic clinical and laboratory features that allow the differentiation of EF from systemic sclerosis and other autoimmune skin diseases, which is useful in everyday clinical practice. The patient complained of increasing swelling of the lower limbs, forearms, and induration in the subcutaneous tissue without involvement of the hands and feet. Based on the biopsy and additional tests, she was diagnosed with EF and treated primarily with oral glucocorticoids (GC). Due to the present side effects of steroid therapy, the decision was made to reduce the doses of GSs with the introduction of methotrexate (MTX). This modification proved successful, and after 23 weeks of treatment, remission of symptoms was observed without further progression of the disease. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to investigate the diagnostic performance of measuring intima-media thickness (IMT) and whole wall thickness (WWT) of deep and superficial veins in the lower extremity of Behcet's disease (BD)., Methods: This cross-sectional study included 67 Behcet patients and 64 healthy control subjects. IMT and WWT of the common femoral (CFV) and great saphenous veins (GSV) were measured by a blinded radiologist using ultrasound. Behcet patients were classified into two groups, namely the vascular and non-vascular groups. The diagnostic performance of the measurements of vein wall thickness in BD was assessed using receiver operating analysis., Results: Bilateral IMT and WWT of the CFV and GSV were significantly higher in BD than in controls (p < 0.01). In the vascular Behcet group, all measurements were also significantly increased compared with the non-vascular Behcet patients (p < 0.01). The area under the curve for all measurements was satisfactory, while CFV WWT indicated the best diagnostic performance [0.82 (0.74-0.89; 95% CI: p < 0.001) for right; 0.81 (0.74-0.88; 95% CI: p < 0.001) for left] for optimal cut-off value (0.595-0.585 mm; sensitivity = 77.3%-77.6%, specificity = 76.2%-76.6% for right and left, respectively). In the regression analysis, older age, vascular presentation, presence of uveitis, and glucocorticoid use were significantly related to vein wall thickness in BD (p < 0.05)., Conclusions: The results of this study provide compelling evidence supporting the use of vein wall thickness (primarily in CFV WWT) as a diagnostic method in BD. Furthermore, the study indicates that the optimal cut-off value of vein wall thickness may vary depending on the characteristics of Behcet cohort., (© 2025 Wiley Periodicals LLC.)

The epidemiology of Moraxella species bloodstream infection (BSI) is poorly defined due to their rarity. We sought to determine the incidence, risk factors, and outcomes of Moraxella species BSI in a large Australian population. All Moraxella species BSIs in patients admitted to Queensland (population estimate 5 million) public health facilities between 2000 and 2019 and submitted to Queensland pathology laboratory-based surveillance were included. Clinical and hospitalisation data were matched with laboratory-based surveillance data. Incidence rate ratios (IRR) with 95% confidence intervals (CI) were calculated. In total, 375 incident Moraxella species BSI occurred during 86 million person-years of surveillance, with an annualised age and sex standardised incidence of 4.3 per million residents. Isolates were most commonly identified as M. catarrhalis (n = 128; 34%) and community-associated (n = 225; 60%). Incidence was highest in infants, with increasing age associated with lower incidence rate. Males were at higher risk (incidence 2.9 vs. 2.0 per million, IRR1.4; 95% CI, 1.2–1.8), this was most pronounced at age extremes. Two-thirds of adults and 43% of children with Moraxella BSI had at least one comorbid illness. When compared to infections in adults, children were more likely to have community-associated disease, and a head and neck source focus of infection. The all-cause 30-day case-fatality rate was 4% (15/375) and this was significantly higher among adults (14/191; 7% vs 1/183; 1%; p < 0.001). Our findings demonstrate the low burden of Moraxella species BSI in a state-wide cohort, for which young children have the highest risk. [ABSTRACT FROM AUTHOR]

Introduction: The pathophysiology of neuropsychiatric manifestations in rheumatoid arthritis is not well known. The magnetic resonance imaging of the brain in rheumatoid arthritis demonstrates non-specific findings, and in the majority of cases, magnetic resonance imaging fails to detect an abnormality, even in rheumatoid arthritis patients with neuropsychiatric manifestations. Therefore, we aimed to assess microstructural integrity changes of white matter in patients with rheumatoid arthritis by using different diffusion tensor imaging parameters. Methods: Eighteen rheumatoid arthritis patients (10 with neuropsychiatric symptoms and 8 without any neuropsychiatric symptoms) and 14 controls were included. The volume of the T2 hyperintense lesions was assessed. Different diffusion tensor imaging parameters such as fractional anisotropy, apparent diffusion coefficient, trace, axial diffusivity, and radial diffusivity were obtained from six different regions of white matter. Inter group significant difference was determined by one-way analysis of variance followed by Tukey's post hoc test. The accuracy of diffusion tensor imaging matrices was evaluated from the receiver operating characteristic curve. Results: No significant difference in lesions' volume was detected between rheumatoid arthritis patients with or without neuropsychiatric symptoms. There was an increased apparent diffusion coefficient and radial diffusivity (p < 0.05) as well as decreased fractional anisotropy and axial diffusivity (p < 0.5) in rheumatoid arthritis patients with neuropsychiatric symptoms compared to controls. Moreover, the apparent diffusion coefficient (p <.05) was increased in both positive and negative MRI of patients with neuropsychiatric features compared to the control group. The sensitivity and specificity of the apparent diffusion coefficient parameters was 73% and 72%, respectively. Conclusions: The various anisotropic metrics were altered in rheumatoid arthritis patients with neuropsychiatric symptoms by using diffusion tensor imaging analysis, representing that central nervous system vasculitis leads to tissue hypoxia resulting in vasogenic edema. This may lead to axonal and myelin degeneration of white matter fibers and neuronal cell disruption. Key Points • Our study confirms that neurovascular events are not uncommon in RA patients with NP features. Diffusion tensor imaging (DTI) is superior to conventional MRI scan for RA patients with NP features because it distinguishes between gray and white matter structures. • RA patients with NP features are more likely to have microstructural changes detected by DTI than by DWI, and it can provide comprehensive anatomical layouts describing regional disparities in neurodegeneration. • DTI's quantitative association of NP symptoms in a large patient cohort is an important study scope. [ABSTRACT FROM AUTHOR]

Cerebral vasculitis is a very rare extra-articular complication of rheumatoid arthritis (RA) that is often challenging to diagnose. Elevated titers of rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP), and antinuclear antibodies (ANA) have been linked with severe complications. The absence of highly elevated titers of RF, anti-CCP, and ANA can complicate the diagnosis of RA-associated cerebral vasculitis. We report the case of a 59-year-old woman with long-standing arthritis maintained on rituximab and leflunomide who developed sudden headaches and altered level of consciousness. Laboratory work-up revealed normal lymphocyte count and mildly elevated total serum protein and anti-CCP with negative RF and ANA and no evidence for viral or bacterial infections. Cerebrospinal fluid analysis (CSF) showed slightly elevated anti-CCP with normal levels of CXCL-13 and interleukin 6 (IL-6). Brain magnetic resonance imaging (MRI) showed ill-defined lesion of high T2 signal. Using MR angiogram, MR perfusion, and MR spectroscopy, the diagnosis of rheumatoid cerebral vasculitis was confirmed. The patient was treated with intravenous methyl-prednisolone with fast complete improvement. We conclude that adequate immunosuppression in RA might not be able to prevent rare extra-articular manifestations such as rheumatoid cerebral vasculitis. [ABSTRACT FROM AUTHOR]

Background: Systemic lupus erythematosus (SLE) and Primary Antiphospholipid Syndrome (PAPS) overlap clinical and immunological features. Therefore, misclassification of PAPS patients as SLE is a concern. The ACR/EULAR 2019 SLE classification has never been studied in PAPS. Objective: To verify if the ACR/EULAR 2019 SLE classification can correctly classify a PAPS patient as not having SLE and compare its performance with the SLICC 2012 SLE classification. Methods: One-hundred thrombotic PAPS patients who fulfilled the Sidney criteria were consecutively screened and those who attended the inclusion criteria were submitted to ACR/EULAR 2019 and SLICC 2012 classifications. Results: Sixty-seven PAPS patients were included in this study. The majority was female (89.6%) with median age at study inclusion of 45 years (35–53) and median PAPS disease duration of 13 years (8–19). PAPS correct classification was observed more often with ACR/EULAR 2019 than SLICC 2021 criteria (94.0% vs. 64.2%; p < 0.001). The 4 misclassified patients in ACR/EULAR 2019 were also misclassified in SLICC 2012. The comparison of misclassified patients to those correctly not classified as SLE resulted, for both criteria, in higher frequencies of hematological domain [ACR/EULAR 2019 (100% vs. 28.6%, p = 0.010) and SLICC 2012 (95.8% vs. 11.6%, p < 0.001)]. Further analysis of hematological manifestations revealed that for the ACR/EULAR 2019 leukopenia (100% vs. 22.2%, p = 0.004) and for the SLICC 2012 leukopenia/lymphopenia (91.7% vs. 7%, p < 0.001) were more frequent in misclassified group. Proteinuria (20.8% vs. 0%, p = 0.004) and low complement (45.8% vs. 20.9%, p = 0.033) were also more often observed in the incorrectly SLICC 2012 classified patients. Conclusion: ACR/EULAR 2019 had high accuracy for distinguishing PAPS from SLE, whereas the SLICC 2012 incorrectly classified more than one third of the PAPS patients as having SLE. [ABSTRACT FROM AUTHOR]

Headaches have been extensively reported in Antiphospholipid syndrome (APS)/Antiphospholipid antibodies (aPL)-positive patients. The aim of this study was to highlight the prevalence of headaches among APS/aPL-positive patients and discuss its association with laboratory, clinical and imaging findings. We searched the literature through Google Scholar and PubMed for publications on the epidemiology, pathogenesis, laboratory, imaging and clinical findings, and management of headaches in APS/aPL-positive patients. The following keywords were used: Antiphospholipid, Hughes syndrome, anticardiolipin, lupus anticoagulant, anti-β2 glycoprotein I, headache, migraine, tension, and cluster. All reports published between 1969 and 2015 were included. Migraine is the most commonly reported type of headache in APS/aPL-positive patients. Thrombotic and platelet dysfunction hypotheses have been studied to uncover the pathogenic role of aPL in the development of headaches. Several studies are reporting higher levels of aPL in primary and secondary APS migraineurs, but only few reached statistical significance. Migraine patients without clinical signs/symptoms of cerebral infarction rarely show positive imaging findings. Digital subtraction angiography shows promise in demonstrating small vascular lesions otherwise not detected on computed tomography, magnetic resonance imaging, or cerebral angiograms. Although it may be solitary and harmless in many cases, the deleterious effect of migraine on the quality of life of APS patients prompts rapid diagnosis and proper management. An anticoagulation trial is advisable in APS patients with migraine as many cases of severe, refractory migraine resolved with anticoagulation therapy. The profile of migraine headaches discussed in this study permits its candidacy for inclusion in future APS classification criteria. [ABSTRACT FROM AUTHOR]

The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports. [ABSTRACT FROM AUTHOR]

Objectives: To gain insight into current methods and practices for the assessment of competences during rheumatology training, and to explore the underlying priorities and rationales for competence assessment., Methods: We used a qualitative approach through online focus groups (FGs) of rheumatology trainers and trainees, separately. The study included five countries-Denmark, the Netherlands, Slovenia, Spain and the United Kingdom. A summary of current practices of assessment of competences was developed, modified and validated by the FGs based on an independent response to a questionnaire. A prioritising method (9 Diamond technique) was then used to identify and justify key assessment priorities., Results: Overall, 26 participants (12 trainers, 14 trainees) participated in nine online FGs (2 per country, Slovenia 1 joint), totalling 12 hours of online discussion. Strong nationally (the Netherlands, UK) or institutionally (Spain, Slovenia, Denmark) standardised approaches were described. Most groups identified providing frequent formative feedback to trainees for developmental purposes as the highest priority. Most discussions identified a need for improvement, particularly in developing streamlined approaches to portfolios that remain close to clinical practice, protecting time for quality observation and feedback, and adopting systematic approaches to incorporating teamwork and professionalism into assessment systems., Conclusion: This paper presents a clearer picture of the current practice on the assessment of competences in rheumatology in five European countries and the underlying rationale of trainers' and trainees' priorities. This work will inform EULAR Points-to-Consider for the assessment of competences in rheumatology training across Europe., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

The prevalence of Type 1 and Type 2 diabetes are increasing significantly worldwide. Whilst vascular complications of diabetes are well recognized, and account for principle mortality and morbidity from the condition, musculoskeletal manifestations of diabetes are common and whilst not life threatening, are an important cause of morbidity, pain and disability. Joints affected by diabetes include peripheral joints and the axial skeleton. Charcot neuroarthropathy is an important cause of deformity and amputation associated with peripheral neuropathy. A number of fibrosing conditions of the hands and shoulder are recognized, including carpal tunnel syndrome, adhesive capsulitis, tenosynovitis and limited joint mobility. People with diabetes are more prone to gout and osteoporosis. Management of these conditions requires early recognition and close liaison between diabetes and rheumatology specialists. [ABSTRACT FROM AUTHOR]

Background Antiphospholipid syndrome ( APS) or 'Hughes syndrome' is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies ( aPL). More than three decades have passed, and experts are still uncovering new pieces of this disease complex pathogenesis and management. Materials and methods We searched in literature using MEDLINE and PubMed databases focusing on the latest development on disease pathogenesis, risk assessment of thrombosis and treatment of APS. Results The phosphatidylinositol 3-kinase ( PI3K)- AKT- mTORC pathway was most recently identified to have a crucial role in activating inflammation among endothelial vessel wall causing vascular lesions in APS. Additionally, new variables are being implemented to assess the risk of thrombosis in patients with APS. Global APS Score ( GAPSS) utilizes cardiovascular risk factors and new autoimmune antibodies as part of the score assessment and is the most valid so far. It can be a promising tool in the future for prediction of thrombosis. Anticoagulation remains the cornerstone in APS; however, many new potential therapeutic agents are developing and are currently under investigation. Conclusions The most recent advances in pathogenesis, risk stratification and treatment provide a platform for high yield studies with the ultimate goal of providing the optimal management to patients with APS. [ABSTRACT FROM AUTHOR]

The author talks about the antiphospholipid (Hughes) syndrome (APS) and presents insights for orthopedics regarding it. Topics discussed include the musculoskeletal complications of APS including arthralgia, necrosis, and osteoporosis; the increased risk of avascular necrosis of bone (AVN) for APS patients; and the use of X-rays and computed tomography (CT) scans for diagnosis.

Multiple sclerosis (MS) and antiphospholipid syndrome (APS) share common clinical, laboratory and radiological features. We reviewed all the English papers on MS and APS published in the literature from 1965 to 2014 using PubMed and Google Scholar. We found that APS can mimic antiphospholipid antibodies (aPL)-positive MS in many ways in its clinical presentation. Nevertheless, APS diagnosis, clinical manifestations and management differ from those of MS. aPL were found in MS patients with titers ranging from 2% to 88%. The distribution and volume of lesions on magnetic resonance imaging (MRI) may help to differentiate MS from primary APS. In addition, atypical MS presentation can guide physicians toward an alternative diagnosis, especially when features of thrombosis and/or history of connective tissue disease are present. In that case, an anticoagulation trial could be worthwhile. [ABSTRACT FROM AUTHOR]