Joint Involvement Research Articles

Epidemiology and Duration of Therapy in Patients with Gram-negative Bloodstream Infections: Retrospective Analysis

Background: Longer courses of antibiotics can be associated with antimicrobial resistance and adverse effects. Randomized clinical trials support treating gram-negative bloodstream infections (GN-BSI) for a shorter duration with a consensus that a seven-day course of antibiotics is appropriate for uncomplicated GN-BSI. Prior to the implementation of a GN-BSI treatment guideline at our institution, we aimed to evaluate the characteristics of patients with GN-BSI and the duration of antibiotic therapy (DOT). Method: We retrospectively reviewed adult inpatients who had a blood culture with at least 1 gram-negative organism within 6 months (November 2022 to April 2023). Patients were excluded if they had a concomitant gram-positive bloodstream infection or if they were transitioned to comfort-focused care within 48 hours of their first positive blood culture. Complicated GN-BSI was defined as exhibiting any of the following: involvement of bone, joint, endovascular system, or foreign body, an inability to achieve source control, immunocompromised status, or failure to demonstrate clinical improvement or culture clearance within 72 hours. The primary outcome of this study was the mean DOT in patients with GN-BSI. Result: 100 patients met the inclusion criteria. Escherichia coli, identified in 54 cases, emerged as the most frequent organism. Urine (41) was the predominant source of bacteremia. Cefepime (48) was the most common empiric agent used. Of the 91 patients with available ceftriaxone susceptibility results, 84% had a susceptible organism. Amongst the 51 patients classified as having a complicated GN-BSI, the leading reason was immunosuppression. Table 1 presents a comparative analysis of complicated vs. uncomplicated GN-BSI. The average DOT for complicated GN-BSI was longer than the uncomplicated infections (20 vs. 11 days, P < 0 .005). Additionally, fewer patients transitioned to oral therapy in the complicated group (33% vs. 67%, P < 0 .005). Conclusion: At our institution, patients with uncomplicated GN-BSI have a shorter DOT and are more likely to transition to oral therapy than those with complicated GN-BSI. However, the mean DOT for uncomplicated infections remained longer than seven days and a large number of uncomplicated GN-BSI patients did not transition to oral therapy, indicating room for improvement in local practice through antimicrobial stewardship initiatives.

Dry Eye Disease in Systemic Lupus Erythematosus

: Systemic lupus erythematosus is a multi-organ autoimmune disease of unknown etiology characterized by widespread inflammation and significant morbidity and mortality. Organ affection includes joint and cutaneous involvement, pulmonary, neurological, and cardiac problems, renal and hematological involvement as well as ocular comorbidities, both posterior but mostly anterior ocular involvement. Therefore, we aimed to evaluate dry eye involvement in lupus patients. A cross-sectional study was performed with the aid of a web-based, anonymous questionnaire that was distributed to both patients diagnosed with lupus erythematosus and to a control group. A total number of 123 patients diagnosed with lupus and 200 responders in the control group completed the questionnaire. Dry eye disease has already been diagnosed in 25 lupus patients (28.9%) compared to 44 (21.9%) individuals in the control group. Dry eye-related symptomatology was reported in 43 (34.60%) patients compared to 57 (28.35%) healthy responders. Upon statistical analysis, more symptoms were reported in the lupus group (p=0.01), in responders diagnosed with dry eye syndrome (p 0.01), and in responders using lubrication (p 0.01) but no association was obtained between the incidence of dry eye symptoms and the presence of the disease. The number of episodes of lupus reactivation since diagnosis was correlated with dry eye syndrome (p=0.15) and the use of lubricating tear drops (p 0.01). Dry eye symptoms and disease are more frequent in lupus patients and further research should be performed to understand the connection between these two disease entities.

First report—parotid tissue choristomas within and adjacent to TMJ ligaments

PurposeTo report the first examples of ectopic salivary glands of TMJ ligaments & capsules. Methods and MaterialsNine subjects with chronic TMJ disorders were found incidentally to have ectopic salivary glands (choristomas) within or near damaged TMJ ligaments and capsules. Clinicopathologic characteristics of 12 TMJs are summarized. ResultsThe 9 subjects were ages 22-64 years (average = 43.0); 6 were female. Microscopic examination revealed major, almost complete ligament replacement by dense, avascular fibrous scar tissue in all but 1 sample. Chronic inflammatory cells were present in the stroma of 3 samples. A total of 33 lobular, independent salivary structures of variable sizes where embedded in or near ligaments and/or capsules. Half were multiple in the same joint; 13 were bilaterally located in the joints of a single subject, with 8 small glandular lobules in one TMJ. All glands consisted of histologically normal serous acini except 1 which showed a focal lobule of mucus acini and another comprised only of mucus acini. Serial sections showed no connection to parotid glands. Some choristomas showed the aging phenomena of fatty infiltrates (n = 7) and oncocytosis (n = 7). Additionally, 3 of the 12 TMJ samples contained preauricular lymph nodes, with 3 nodes in 1 joint and bilateral involvement in 1 subject. Of the 5 identified nodes, all but one was hyperplastic, with nonspecific inflammatory histiocytosis. ConclusionsWe report, for the first time, independent salivary gland choristomas (n = 33) within the soft tissues of the TMJ (n = 12), with one joint containing 8 such choristomas. All consisted of normal serous tissue except one, a mucus gland. Additionally, a fourth of TMJ surgical samples contained hyperplastic preauricular lymph nodes.

New Insight into the Effects of Different Types of Midfoot and Hindfoot Deformities on the Functional Outcomes of Rheumatoid Arthritis Patients

In advanced stages of rheumatoid arthritis (RA), the pattern of joint involvement leads to varied types of foot deformities such as forefoot, midfoot, and hindfoot deformities. We aim to evaluate midfoot and hindfoot deformities and their effect on functional outcomes in RA patients. In this prospective cross-sectional study clinical and radiological evaluations of 100 adult RA patients were studied for midfoot and hindfoot deformities. The RA disease activity, functional disability level, specific foot and ankle assessments, gait speed, and balance tests were assessed. Radiological examinations of the weight-bearing feet in a standing position were done. We found that the most frequent deformity type was the planovalgus foot (24%). There were statistically significant differences between the types of foot deformities and disease duration, disease activity, falls, Tinetti and Berg balance Test, and health assessment questionnaire-disability index. The multivariate regression analysis of independent correlates of falling detected that patients with equinovarus deformity were 2.5 times more liable for falling with 63.5% predictive power. According to the predictive criteria of deformity type for falling, the equinocavovarus type had accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 91%, 84.5%, 98.5%, 99%, and 84%, respectively. We found in RA patients, midfoot and hindfoot deformities cause a significant reduction of functional ability and quality of life. The early detection of foot deformities requires a good awareness of the clinical and radiographic diagnosis of different types of deformities thus reducing the possible functional disabilities via early management by combined orthotic, physiotherapy, and surgical treatments.

Rheumatoid Arthritis - Symptoms and Treatment Demystified

Rheumatoid Arthritis is characterized by the body's immune system mistakenly attacking its own tissues, particularly the synovium, which lines the joints. It commonly presents joint pain, swelling, stiffness, and reduced mobility, which affect multiple joints symmetrically. Beyond joint involvement, RA can impact various organs and systems throughout the body, leading to symptoms like fatigue, fever, and weight loss. Early diagnosis and aggressive treatment can slow down disease progression and mitigate joint damage. While there's no cure, numerous treatment modalities exist to alleviate symptoms, reduce inflammation, and maintain joint function.

Mendelian randomization based on genome-wide association studies and expression quantitative trait loci, predicting gene targets for the complexity of osteoarthritis as well as the clinical prognosis of the condition.

Osteoarthritis (OA) entails a prevalent chronic ailment, marked by the widespread involvement of entire joints. Prolonged low-grade synovial inflammation serves as the key instigator for a cascade of pathological alterations in the joints. The study seeks to explore potential therapeutic targets for OA and investigate the associated mechanistic pathways. Summary-level data for OA were downloaded from the genome-wide association studies (GWAS) database, expression quantitative trait loci (eQTL) data were acquired from the eQTLGen consortium, and synovial chip data for OA were obtained from the GEO database. Following the integration of data and subsequent Mendelian randomization analysis, differential analysis, and weighted gene co-expression network analysis (WGCNA) analysis, core genes that exhibit a significant causal relationship with OA traits were pinpointed. Subsequently, by employing three machine learning algorithms, additional identification of gene targets for the complexity of OA was achieved. Additionally, corresponding ROC curves and nomogram models were established for the assessment of clinical prognosis in patients. Finally, western blotting analysis and ELISA methodology were employed for the initial validation of marker genes and their linked pathways. Twenty-two core genes with a significant causal relationship to OA traits were obtained. Through the application of distinct machine learning algorithms, MAT2A and RBM6 emerged as diagnostic marker genes. ROC curves and nomogram models were utilized for evaluating both the effectiveness of the two identified marker genes associated with OA in diagnosis. MAT2A governs the synthesis of SAM within synovial cells, thereby thwarting synovial fibrosis induced by the TGF-β1-activated Smad3/4 signaling pathway. The first evidence that MAT2A and RBM6 serve as robust diagnostic for OA is presented in this study. MAT2A, through its involvement in regulating the synthesis of SAM, inhibits the activation of the TGF-β1-induced Smad3/4 signaling pathway, thereby effectively averting the possibility of synovial fibrosis. Concurrently, the development of a prognostic risk model facilitates early OA diagnosis, functional recovery evaluation, and offers direction for further therapy.

Is there any difference between M694V heterozygote and non-exon 10 mutations on symptoms onset and response to colchicine treatment?

Purpose: Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory syndrome throughout the world. The most frequent genotype-phenotype correlation is in a certain part of exon 10, especially M694V mutation. There are also a group of patients with non-exon 10 mutations, who have a similar clinical spectrum of the disease. We aim to investigate the genotype-phenotype differences between M694V heterozygote mutations and non-exon 10 mutations. Materials and methods: Data charts of children (n=431) with FMF from two tertiary hospitals were reviewed. Patients were divided into two groups with regard to having M694V heterozygote or non-exon 10 mutations. Genotype-phenotype features and response to treatment were compared. Results: There were M694V heterozygote mutations in 128 (29.7%) patients and non-exon 10 mutations in 303 (70.3%) patients. The follow-up period was 54.5 (33-105) months. There was no difference between the age of symptoms onset, the age of diagnosis, and the diagnosis delay time. The family history in patients with M694V heterozygote mutation was statistically positive compared to non-exon 10 mutation group (p=0.001). The symptoms of joint involvement as arthritis and PRAS scores were significantly higher in the M694V heterozygote group (p=0.026 and p=0.001). Additionally, biological agent need due to colchicine unresponsiveness was statistically higher in M694V heterozygote group than group with non-exon 10 mutation (p=0.004). Conclusion: There is a significant difference between children with M694V and non-exon 10 mutations, even when the M694V mutation is present in one allele only. Family history with FMF, musculoskeletal symptoms, and unresponsiveness to colchicine are main parameters.

Ultrasound imaging for sternoclavicular joint involvement in enthesitis-related arthritis: A closer look at capsular enthesitis

Introduction: Enthesitis-related arthritis is a specific subtype of juvenile idiopathic arthritis characterised by the co-presence of arthritis and enthesitis or the evidence of one of them coupled with at least two among sacroiliac joint tenderness, inflammatory back pain, presence of human leukocyte antigen-B27, acute symptomatic anterior uveitis, onset in a male child aged 6 years or older or history of spondyloarthropathy in a first-degree relative. Small joints like the sternoclavicular joint are rarely affected, and the prevalence of their involvement in enthesitis-related arthritis has been poorly assessed in the pertinent literature. Case report: The authors report an atypical case of left sternoclavicular joint arthritis/enthesitis in a 12-year-old male child with juvenile idiopathic arthritis. The B-mode sonographic findings of articular effusion, synovial hypertrophy and capsular bulging, coupled with the microvascular mapping with colour Doppler of the sternoclavicular joint, have been crucial to optimising the pharmacological approach in clinical practice. In this sense, the sonographic examination presented as a natural extension of the physical examination to accurately define the poor control of disease activity using first-line pharmacological agents. Discussion: The present case report can be considered the first to accurately report the B-mode and the colour Doppler findings of a pathological sternoclavicular joint in a patient enthesitis-related arthritis. Ultrasound imaging demonstrated intra-articular effusion, capsular bulging and synovial hypertrophy clearly defining sternoclavicular joint arthritis. Interestingly, the hypervascularisation involved both the synovial tissue and the capsule–bone interface – that is, the enthesis of the joint capsule – suggesting a potential co-existence of arthritis and enthesitis of the sternoclavicular joint in enthesitis-related arthritis patients. Conclusion: The high-resolution point-of-care ultrasound seems to be a potential ‘game changer’ in paediatrics to promptly optimise the pharmacological management in enthesitis-related arthritis patients. Indeed, unlike other imaging modalities such as magnetic resonance imaging, modern high-frequency ultrasound transducers guarantee a superior spatial resolution of superficial joints and allow an accurate mapping of small-size and low-flow vascular elements of synovial and capsular tissues optimising the grading of disease activity and avoiding the use of contrast agent.

Application of a natural language processing artificial intelligence tool in psoriasis: A cross-sectional comparative study on identifying affected areas in patients’ data

Psoriasis is an immune-mediated skin disease affecting approximately 3% of the global population. Proper management of this condition necessitates the assessment of the body surface area and the involvement of nails and joints. The integration of natural language processing with electronic medical records (EMRs) has recently shown promise in advancing disease classification and research. This study evaluates the performance of ChatGPT-4, a commercial artificial intelligence platform, in analyzing unstructured EMR data of psoriasis patients, particularly in identifying affected body areas. The study analyzed EMR data from 94 patients treated at the Dermatology Department and Psoriasis Outpatient Clinic of Sheba Medical Center between 2008 and 2022. The data were processed using the ChatGPT-4 interface to identify and report the body areas affected by psoriasis. These identified areas were then categorized, and the accuracy of ChatGPT-4’s analysis was compared with that of a senior dermatologist. The results revealed that the dermatologist identified 477 psoriasis-affected body areas. ChatGPT-4 accurately recognized 443 (92.8%) of these areas, missed 34, and incorrectly identified 30 areas as affected. From 94 cases, nail involvement was detected in 32 cases (34.0%), with ChatGPT-4 correctly identifying 29 cases. Joint involvement was noted in 25 cases (26.6%), with 24 correctly identified using ChatGPT-4. Complete accuracy was achieved in 54 cases (57.4%), although inaccuracies were observed in 40 cases (42.6%). We found that cases with more characters, words, or identified body areas were more prone to errors, suggesting that increased data complexity heightens the likelihood of inaccuracies in artificial intelligence analysis. ChatGPT-4 demonstrated high performance in analyzing detailed and complex unstructured EMR data from patients with psoriasis, effectively identifying involved body areas, including nails and joints. This highlights the potential of NLP algorithms to enhance the analysis of unstructured EMR data for both clinical follow-up and research purposes.

Joint involvement in patients with acromegaly: potential markers for early diagnosis

Joint involvement in patients with acromegaly: potential markers for early diagnosis

The Lung in Rheumatoid Arthritis-Friend or Enemy?

Rheumatoid arthritis (RA) is a chronic autoimmune condition frequently found in rheumatological patients that sometimes raises diagnosis and management problems. The pathogenesis of the disease is complex and involves the activation of many cells and intracellular signaling pathways, ultimately leading to the activation of the innate and acquired immune system and producing extensive tissue damage. Along with joint involvement, RA can have numerous extra-articular manifestations (EAMs), among which lung damage, especially interstitial lung disease (ILD), negatively influences the evolution and survival of these patients. Although there are more and more RA-ILD cases, the pathogenesis is incompletely understood. In terms of genetic predisposition, external environmental factors act and subsequently determine the activation of immune system cells such as macrophages, neutrophils, B and T lymphocytes, fibroblasts, and dendritic cells. These, in turn, show the ability to secrete molecules with a proinflammatory role (cytokines, chemokines, growth factors) that will produce important visceral injuries, including pulmonary changes. Currently, there is new evidence that supports the initiation of the systemic immune response at the level of pulmonary mucosa where the citrullination process occurs, whereby the autoantibodies subsequently migrate from the lung to the synovial membrane. The aim of this paper is to provide current data regarding the pathogenesis of RA-associated ILD, starting from environmental triggers and reaching the cellular, humoral, and molecular changes involved in the onset of the disease.

Evaluation of temporomandibular joint morphology and morphometry in male osteoporotic patients using advanced imaging and biochemical markers: A cross-sectional study.

Osteoporosis is a condition with reduced bone mass and disrupted architecture. Osteoporosis affects the Temporomandibular disorders (TMD) by changing bone density and quality. This study aims to determine the nature and extent of temporomandibular joint (TMJ) involvement in osteoporotic patients by correlating TMJ morphological changes detected by CBCT with systemic bone health indicated by BMD T-scores from DEXA and analyzing BTMs in serum and saliva. This study was a cross-sectional study conducted from May 2021 to December 2022. It involved 50 participants divided into two groups (N=25). One group was healthy male, while the other group had osteoporosis male. Saliva and blood samples were collected, and diagnostic imaging was conducted. The prevalence of various bone changes in the condyle was examined using CBCT. Erosion was found to be the most common, followed by Flattening, Osteophyte, and Subchondral cysts. The study group had significantly higher rates of smooth condyle, erosive lesions, and osteophytes compared to the control group. Pseudocyst decreased on the right side but increased on the left side. Pain on the right side increased more in the study group, and the T score for osteoporosis was higher in the study group. Joint spaces, condyle diameter, and glenoid cavity measurements differed significantly between sick and healthy people, as shown by CBCT (P≤0.001). Only the ALP parameter in the serum showed a significant increase in the study group compared to the control group. Saliva analysis revealed higher levels of calcium, osteocalcin, and ALP in the case group compared to the control group. The results of this study showed that CBCT as a specialized technique in imaging by providing detailed images can be used to evaluate osteoporosis and be used as an accurate diagnostic tool.

Immunoglobulin A vasculitis: The clinical features and pathophysiology.

Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild proteinuria to severe nephritic or nephrotic syndrome highlights the importance of monitoring kidney function in patients with IgAV. Recognizing these key features is crucial for early diagnosis and appropriate management to prevent long-term complications related to kidney disease. However, the pathogenesis of IgAV remains unclear. Disease mechanisms involve various factors, including the interplay of aberrantly glycosylated IgA, anti-endothelial cell antibodies, and neutrophils following infection triggers, which are the main pathogenic mechanisms of IgAV. Insights from cases of IgAV related to Coronavirus disease 2019 have offered additional understanding of the connection between infection and IgAV pathogenesis. This review provides a valuable resource for healthcare professionals and rheumatology researchers seeking a better understanding of the clinical features and pathophysiology of IgAV.

Review study of Psoriasis in Iraq

Abstracts are written in Indonesian and English. Describe the introduction, the purpose of writting, research methods, result and conclusion. Minimum 150 and maximum 250 words.. Psoriasis is a multisystem inflammatory disease with predominantly skin and joint involvement. Beyond the physical dimensions of disease, psoriasis has an extensive emotional and psychosocial effect on patients, affecting social functioning and interpersonal relationships. As a disease of systemic inflammation, psoriasis is associated with multiple comorbidities, including cardiovascular disease and malignancy. The diagnosis is primarily clinical and a skin biopsy is seldom required. Depending on the severity of disease, appropriate treatment can be initiated. For mild to moderate disease, first-line treatment involves topical therapies including corticosteroids, vitamin D3 analogues, and combination products. These topical treatments are efficacious and can be safely initiated and prescribed by primary care physicians. Patients with more severe and refractory symptoms might require further evaluation by a dermatologist for systemic therapy .Psoriasis is a chronic, noncommunicable, painful, disfiguring and disabling disease for which there is no cure and with great negative impact on patients’ quality of life . It can occur at any age. The etiology of psoriasis remains unclear. Psoriasis can also be provoked by external and internal triggers, including mild trauma, sunburn, infections, systemic drugs and stress . Psoriasis involves the skin and nails, and is associated with a number of comorbidities. Skin lesions are localized or generalized, mostly symmetrical, sharply demarcated, red papules and plaques, and usually covered with white or silver scales. Lesions cause itching, stinging and pain. Individuals with psoriasis are reported to be at increased risk of developing other serious clinical conditions such as cardiovascular and other noncommunicable diseases . Psoriasis causes great physical, emotional and social burden . Disfiguration, disability and marked loss of productivity are common challenges for people with psoriasis. Topical and systemic therapies as well as phototherapy are available.

Psoriasis knowledge gaps and misconceptions in the Middle East and North Africa (MENA) regions: patients' survey-based study.

Misconceptions among psoriatic patients often lead to a negative impact on disease outcomes. Our main target was knowledge assessment among a sample of psoriatic patients in the Middle East and North Africa (MENA) region where data are scarce. The present study is a cross-sectional descriptive survey. It consists of an online questionnaire comprising 19 questions designed to assess psoriasis knowledge and five demographic questions. The questionnaire link was posted on the official Facebook page of the Kasr Al Ainy Psoriasis Unit (KAPU). The questionnaire was taken by 527 participants, but only 396 responses were complete and adequate for analysis. The mean psoriasis knowledge score was higher in females (P = 0.005) and participants with advanced education degrees (P < 0.001). Patients reporting regular follow-ups with dermatologists were more likely to acknowledge joint involvement (P = 0.044) but also incorrectly assume biologics are a final cure (P = 0.038). In addition, they were more likely to assume psoriasis affects pregnancy (P = 0.013). Patients with a family history of psoriasis showed a better mean knowledge score than those without (P = 0.01). Only 54.55% of participants reported knowledge of possible disease exacerbation by drugs. A minority (26.77%) of our patients responded that a diet change could not permanentlycure psoriasis. This study reports knowledge gaps in a cohort of Arabic-speaking psoriasis patients, especially regarding areas of extracutaneous involvement, the hereditary nature of the disease, and the effect of psoriasis on pregnancy and fertility. Most participants were unaware that biological therapy and a change in diet do not offer a permanent cure. Dermatologists in our region must reach out to their patients and correct the various misconceptions reported in this study.

A case of multiple myeloma misdiagnosed as seronegative rheumatoid arthritis: a rare case report

Introduction: Multiple myeloma (MM) is a malignant plasma cell proliferation producing large numbers of monoclonal immunoglobulins. Typical MM symptoms include anemia, bone pain, hypercalcemia, and renal failure. Atypical presentations like joint involvement were rarely reported in the literature and may cause significant delays in treatment and adverse outcomes. Case presentation: The authors report a case of a 54-year-old female who presented with symmetrical polyarthritis and was misdiagnosed with rheumatoid arthritis. The diagnosis of MM was made after failing many treatments of rheumatoid arthritis and with further laboratory tests and procedures. Conclusion: This rare manifestation of MM carries a diagnostic challenge and causes a significant delay in treating such patients. Here, the authors report this unusual initial presentation with a review of several cases in the literature describing similar presentations.

Long-term follow-up of corrective jaw surgery including distraction osteogenesis in 32 patients with juvenile idiopathic arthritis

Dentofacial deformity following juvenile idiopathic arthritis (JIA) with temporomandibular joint (TMJ) involvement is associated with functional, aesthetic, and psychosocial impairment. Corrective surgical treatment includes combinations of orthognathic surgeries (OS). The aims of this study were to assess orofacial symptoms, functional and aesthetic status, and stability after OS including mandibular distraction osteogenesis (MDO). A prospective study was conducted of 32 patients with JIA of the TMJ and dentofacial deformities who underwent MDO as the only surgery or in combination with bilateral sagittal split osteotomy, Le Fort I, and/or genioplastybetween 2003 and 2018. Data from clinical examinations and cephalograms performed pre- and postoperative and at long-term (mean 4 years) were analysed. Patients experienced unchanged orofacial symptoms (all P > 0.05), short-term TMJ functional impairment (all P < 0.001), and long-term morphological improvements in SNB angle (P < 0.001), anterior facial height (P < 0.001), mandibular length (P = 0.049), overjet (P < 0.001 and P = 0.005), and posterior facial symmetry (P = 0.046). MDO as the only surgery or with secondary adjunctive OS improved dentofacial morphology in terms of mandibular advancement, anterior facial height, posterior facial symmetry, and incisal relationships without long-term deterioration in TMJ function or orofacial symptoms.

Gender disparities in Behçet's syndrome: identifying distinct phenotypes through cluster analysis.

Behçet's syndrome (BS) is a complex, multi-systemic disorder with a global occurrence, notably concentrated along the Silk Road. This study aimed to investigate gender-specific expressions and clinical phenotypes in BS patients within the Eastern Black Sea Region of Turkey. A total of 290 BS patients were retrospectively analyzed between January 2013 and December 2023. Demographic characteristics, clinical manifestations, medical treatment, and pathergy test results were obtained from a review of medical records. The mean age was 45.79 ± 13.05, with a male-to-female ratio of 48.6:51.4. Male patients had higher papulopustular lesions (p < 0.001) and ocular involvement (p = 0.036), while females showed more frequent genital ulcers (p = 0.032). Medication usage showed gender-based variations, notably higher corticosteroid, azathioprine, and tumor necrosis factor-alpha inhibitor (TNFi) use in males (p < 0.001). Cluster analysis revealed five distinct clusters, each with unique features and gender predominance. Cardiovascular type, ocular type, and skin type predominantly featured male patients, while joint involvement type and neurologic and mucosal involvement type were more prevalent among female patients with BS. This research contributes valuable insights into the gender-related clinical variations of BS within a specific geographic region, fostering a more comprehensive understanding of this challenging syndrome. The identification of distinct clinical phenotypes facilitates the development of tailored treatment strategies, potentially leading to improved outcomes for patients with BS.

Markers associated with the development of osteoporosis in children with juvenile idiopathic arthritis

Background. The purpose of the study was to investigate the association between bone mineralization le­vel in children with juvenile idiopathic arthritis (JIA) and various exogenous and endogenous factors. Materials and ­me­thods. The study group consisted of 162 children diagnosed with JIA. Medical history, clinical features, and treatment of JIA were studied. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score (JADAS-27), health status — with the Childhood Health Assessment Questionnaire (CHAQ). Complete blood count with erythrocyte sedimentation rate, biochemical analysis (C-reactive protein, total and ionized calcium) were performed, and instrumental methods (ultrasound densitometry, X-ray and ultrasound examination of joints) were used. Serum levels of osteocalcin, 25-hydroxyvitamin D (25(OH)D), and parathyroid hormone were evaluated using an enzyme-linked immunosorbent assay on an ER500 analyzer (Sinnowa, China). Results. According to our study, osteoporosis was observed in 19 patients (11 girls and 8 boys) out of 162 children with JIA, accounting for 11.7 %. It was significantly more frequent in patients with polyarthritis (73.7 %) compared to those with oligoarthritis (26.3 %) (p &lt; 0.05). Conclusions. Osteoporosis can develop in children with JIA who do not receive systemic glucocorticoid therapy. The main markers associated with osteoporosis in children with JIA include non-infectious disease onset trigger, cervical joint involvement at disease onset, involvement of more than 3 joint groups at onset, gait disturbances, high JADAS-27 and CHAQ scores, signs of systemic inflammatory process, including elevated erythrocyte sedimentation rate.

A specific diagnostic metabolome signature in adult IgA vasculitis

IntroductionIgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker.ObjectiveWe aimed to explore the baseline serum metabolome of adult patients with IgA vasculitis to identify potential diagnostic biomarkers.MethodsWe performed a study comparing the serum metabolome of patients with IgA vasculitis to that of patients with inflammatory condition, namely spondyloarthritis. Serum analyses were performed by high-performance liquid chromatography-mass spectrometry.ResultsFifty-five patients with IgA vasculitis and 77 controls with spondyloarthritis (age- and sex-matched) were included in this study. The median age of IgA vasculitis patients was 53 years. Two-thirds of patients were female (n = 32). At the time of vasculitis diagnosis, 100% of patients had skin involvement and 69% presented with glomerulonephritis (n = 38). Joint and digestive involvement were observed in 56% (n = 31) and 42% (n = 23) of patients. Four discriminative metabolites between the two groups were identified: 1-methyladenosine, L-glutamic acid, serotonin, and thymidine. The multivariate model built from the serum metabolomes of patients with IgA vasculitis and spondyloarthritis revealed an accuracy > 90%. As this model was significant according to the permutation test (p < 0.01), independent validation showed an excellent predictive value of the test set: sensitivity 98%; specificity 98%, positive predictive value 97% and negative predictive value 98%.ConclusionTo our knowledge, this study is the first to use the metabolomic approach for diagnostic purposes in adult IgA vasculitis, highlighting a specific diagnostic metabolome signature.