Rheumatology International最新文献

We aimed to assess the typical experiences, desired outcomes, satisfaction with clinical and anticipated outcomes, and the importance of improvements for individuals with Hypermobile Ehlers-Danlos Syndrome (hEDS) and Generalized Hypermobility Spectrum Disorder (G-HSD). A cross-sectional survey was conducted among adults aged 18 and above with hEDS and G-HSD. The survey included the Patient-Centered Outcome Questionnaire and an adapted version addressing common concerns in these individuals. Descriptive statistics were used for analysis. The survey received 483 responses with an 82% completion rate. Most respondents were females (90%), aged 21-30 (30%), living in North America (76%), and diagnosed with hEDS (80%). Participants diagnosed with hEDS reported higher typical levels of pain compared to those diagnosed with G-HSD and higher expected levels of pain and interference with daily activities post-treatment (p < 0.05). The areas of most significant concern were pain, fatigue, interference with daily activities, and walking issues. Our findings revealed no differences in how individuals from both groups rated their treatment expectations, except for the usual pain level and the expected pain level and interference with daily activities post-treatment. Patients' perspectives are essential for developing appropriate treatment plans and improving outcomes for this patient population. Our results will hopefully inform the development of new interventions to impact outcomes that matter to individuals with hEDS and G-HSD.

Introduction: Temporomandibular joint (TMJ) septic arthritis is a rare frequently misdiagnosed condition with non-specific symptoms. We present our experience of thirteen cases of TMJ septic arthritis and perform a systematic review of the literature to collate the multiple characteristics of this condition.

Material and method: A total of 133 cases of TMJ septic arthritis in humans across 62 studies were analyzed by searching PubMed, Cochrane Library, DOAJ and ClinicalTrials.gov using the following search terms: "TMJ septic arthritis," "Temporomandibular septic arthritis," "TMJ infectious arthritis," and "Temporomandibular infectious arthritis."

Results: We identified three routes of TMJ septic arthritis dissemination: the hematogenous route, direct inoculation, and local contiguity. Joint and rheumatic pathologies and immunomodulatory diseases are risk factors. The most frequently causative bacterial genus is Staphylococcus, followed by Streptococcus. Causative bacteria can be identified by bacteriological analysis. Magnetic resonance imaging, computed tomography (CT), and scintigraphy can be used for diagnosis, but CT is the gold standard in an emergency setting. Blood tests often reveal a high C-reactive protein concentration and high leukocyte counts. Signs and symptoms include preauricular swelling and trismus, and, less commonly, fever, ipsilateral hemifacial pain, joint disorder, and malocclusion with mandibular deviation. Timely treatment is key to avoid short and long-term complications, because proteolytic enzymes from granulocytes can cause irreversible damage within 7 days. Antibiotic therapy, arthroplasty, and physiotherapy are commonly used treatment modalities.

Conclusion: TMJ septic arthritis can be misdiagnosed due to its non-specific clinical manifestations. Complications can occur; thus, timely and effective treatment is key.

To investigate if progression of coronary artery calcification (CAC) in patients with systemic lupus erythematosus (SLE) is associated with renal and traditional cardiovascular risk factors as well as incidence of myocardial infarctions. CAC progression was evaluated by cardiac computed tomography (CT) at baseline and after 5 years. Multivariable Poisson regression was applied to investigate associations between CAC progression and baseline values for traditional cardiovascular risk factors, CAC, SLE disease duration, lupus nephritis, and renal function. Regarding renal function, three groups were defined based on eGFR. Further, we analysed association between CAC progression and myocardial infarction during follow-up. Of the 147 SLE patients, 99 had cardiac CT at baseline and 5-year follow-up, with a total of 502 patient-years. At baseline, their median age was 47 years, median SLE disease duration was 14 years, 88% were women, 58% had lupus nephritis, and the median eGFR was 99 mL/min/1.73m². 38/99 (39%) had CAC progression. CAC progression was associated with smoking (ever) (relative risk [RR] 1.69, CI95% 1.19-2.40), SLE disease duration (RR per year 1.03, CI95% 1.01-1.04), and CAC presence (RR 2.52, CI95% 1.68-3.78) at baseline. During follow-up, myocardial infarction occurred in three (7.9%) CAC progressors and in two (3.3%) patients who did not have CAC at any time (RR 2.1, CI95% 0.0-5.5). In this study, progression of CAC was associated with smoking, SLE disease duration and the prior presence of CAC, but it was inconclusive as to associations with renal involvement and incidence of MI.

This study investigated severity, course and patterns of fatigue surrounding subcutaneous biological disease-modifying antirheumatic drug (bDMARD) injection in inflammatory rheumatic disease (IRD) patients using ecological momentary assessments and investigated self-reported adverse drug reactions (ADRs). In this prospective cohort study, IRD patients completed fatigue severity numeric rating scales (0-10) in web-based ecological momentary assessments in three waves of five days surrounding bDMARD injection. The course of fatigue was measured by the change in fatigue from pre-dosing to post-dosing scores and was classified as: worsening, improving or no clinically relevant change. A pattern was defined as a course of worsening, improving or no clinically relevant change in fatigue in at least two out of three waves for patients completing assessments across all three waves. ADRs could be reported on day five of each wave. In total 609 participants completed ecological momentary assessments surrounding 1541 bDMARD injections. Overall average fatigue severity across all three waves was 4.5 (± SD 2.4) and 78% experienced severe fatigue in at least one assessment. Of 398 patients completing all three waves, 61% had no clinically relevant change in fatigue in at least two out of three waves, 13% had a pattern of worsening fatigue and 18% had a pattern of improving fatigue. Of 398 patients, 36% had a consistent pattern in all three waves. IRD patients using a bDMARD may consistently experience specific fatigue patterns surrounding bDMARD administration. These patterns provide insights for clinical practice and could be used to inform patients properly.

Background: Psoriatic arthritis (PsA) significantly contributes to increased morbidity, reduced life expectancy, and higher healthcare costs due to the burden of comorbidities. This study assessed the prevalence of comorbidities in PsA patients in India and explored the influence of age and disease duration on these comorbidities.

Methods: The prospective, multicenter observational study was conducted across seven centers in India, utilizing data from the Indian Rheumatology Association. Data were collected using expert-validated proformas, with comorbidities classified according to the ICD-10 Charlson Comorbidity Index. Participants were divided into two age groups: <40 years and ≥ 40 years.

Results: The study included 533 participants (median age: 50 years, range: 17-81). Comorbidities were more common among older patients (median age: 54 years, range: 41-79). About 38% had at least one comorbidity, with hypertension (17.07%) and diabetes (15.19%) being most prevalent, followed by thyroid disorders (10.13%) and hyperlipidemia (3.94%). Comorbidities were significantly more common in patients ≥ 40 years (45.04%) compared to those < 40 years (18.57%) (P < 0.001). The older group also had higher rates of hyperlipidemia (4.83% vs. 0.71%, P = 0.035) and hypertension (22.14%). Logistic regression revealed a significant association between age and the prevalence of diabetes, hypertension, and thyroid disorders (P = 0.003). Among patients over 40, 44.27% had at least one comorbidity.

Conclusion: The study underscores the significant association between age and the prevalence of comorbidities in PsA patients, particularly in those over 40 years. These findings highlight the importance of targeted screening and management in older PsA patients.

Scleromyxedema is a rare chronic fibromucinous disorder characterized by a generalized papular and sclerodermoid eruption. Despite its clinical significance, no definitive therapeutic guidelines exist for scleromyxedema, making management challenging. Herein, we present a case of a 76-year-old female patient referred for evaluation of systemic sclerosis, presenting with distinctive cutaneous manifestations and neurological symptoms. Investigations revealed monoclonal gammopathy (IgG Lambda subtype) and antinuclear antibodies, supporting a diagnosis of scleromyxedema. A multidisciplinary approach with intravenous immunoglobulin (IVIG) therapy resulted in significant improvement in neurological and cutaneous symptoms. This case underscores the challenges of diagnosing and managing scleromyxedema and highlights IVIG as a potential therapeutic option in the absence of standardized guidelines.

Gastrointestinal (GI) involvement is highly prevalent in systemic sclerosis (SSc) and significantly affects patient quality of life and clinical outcomes. This study investigates the potential of undernutrition scores, namely the Control of Nutritional Status (CONUT) score and the Prognostic Nutrition Index (PNI), in predicting GI involvement in patients with SSc. A total of 82 patients diagnosed with SSc were enrolled in this cross-sectional study. Participants were evaluated using the UCLA Scleroderma Clinical Research Consortium Gastrointestinal Tract 2.0 (UCLA GIT 2.0) tool, which assesses the severity of GI symptoms and their impact on health-related quality of life. Malnutrition was assessed using CONUT and PNI scores derived from routine laboratory parameters. The correlation between these malnutrition indices and the UCLA GIT 2.0 scores was analyzed to determine the predictive value of malnutrition in GI involvement. The study found that patients with higher CONUT scores, indicating malnutrition, had significantly higher total UCLA GIT 2.0 scores. A moderate positive correlation was observed between CONUT scores and total UCLA GIT 2.0 scores (r =.539; p <.01), while a negative correlation was found between CONUT scores and PNI (r = -.513; p <.01). These findings suggest that malnutrition, as measured by CONUT and PNI, is associated with greater GI involvement in SSc. This study shows that malnutrition indices such as CONUT and PNI in SSc patients, together with the UCLA GIT 2.0 score, may serve as usefull predictors of GI involvment in routine clinical practice.

Idiopathic granulomatous mastitis (IGM) is a rare inflammatory disease of the breast. Various clinical management approaches have been described, but their efficacy and optimal sequential order remain uncertain. We describe the first Canadian cohort of patients with IGM, discuss treatment outcomes and outline a practical management approach. This retrospective study included patients diagnosed with biopsy-confirmed IGM between 2014 and 2023, aged over 18 years. Based on a scoping review of the literature, a diagnostic and management approach was developed, and we present here the disease course and outcomes using this approach. 22 females were included, with a mean age of 40 (24-65) years, mostly presenting with a breast lump (n =22, 100%) and breast pain (n = 15, 68%). Mean rheumatology follow-up was 28.7 months (range 3-79). Mean time from first symptom to diagnosis was 3.5 months (range 1-13). Corynebacterium kroppenstedtii was found in 8 patients. Treatment including lipophilic antibiotics, corticosteroids and disease modifying anti-rheumatic drugs (DMARDs), led to complete remission in 95% of patients, in a mean time of 11.6 months (range 1-36), and relapse in only 1 patient. 11 patients required DMARDs (50%), most commonly methotrexate (n=9). We highlight the variable severity of IGM and the benefits of a severity-based treatment approach. A diligent evaluation and work-up is essential to manage IGM. The proposed severity-based management approach with medical treatment and less aggressive surgical intervention led to complete remission in 95%.

To examine changes in symptoms and health status in patients with fibromyalgia (FM) 24 months after participating in the mindfulness-based group-program, the Vitality Training (VTP), followed by physical exercise counselling. Seventy-six participants, mean age (range) 43 (26-52), females 69 (91%), diagnosed with FM according to the ACR 2011-criteria received the VTP in a previous randomised controlled trial. Control group participants could receive the VTP after a 12-month observation period, therefore only data from the intervention group were analysed in the present study. Self-reported data were collected electronically at baseline, 3, 12 and 24 months. Outcomes were patient global impression of change (PGIC), FM-severity, i.e. widespread pain (WPI) and symptom severity (SSS), pain, fatigue, sleep quality, psychological distress, motivation and barriers for physical activity, mindfulness and work participation. Trends across time-points were analysed using mixed models for repeated measurements. At 24-months, 48 (56.5%) participants responded, 94% female, median (range) age 46 (28-54), symptom duration 12 (5-33) years. Seven participants reported much/very much better on the PGIC; 21 (44%) reported no change/minimal improvement. Improvements were observed in WPI (-1.9, ES 0.4), SSS (-1.2, ES 0.6), fatigue (-0.8, p =.014) and self-efficacy for physical activity (1.4, ES 0.4). There was a significant trend of reduced WPI, SSS, pain and fatigue across the four time-points, but no additional improvements from 12 to 24-month follow-up. Participants who had completed the VTP demonstrated small to moderate improvements in symptom burden and FM-severity from baseline to 24-month follow-up. Trial registration number: ISRCTN96836577 https://doi.org/10.1186/ISRCTN96836577 , prospectively registered 12.07.2016.

Chimeric antigen receptor T-cell (CAR-T) therapy has revolutionized the treatment of various hematological malignancies. Recently, CAR-T has been used in refractory auto-immune diseases with initial encouraging results. In this systematic review, we examined the safety and efficacy of CAR-T in patients with refractory auto-immune diseases. PubMed/Medline, EMBASE, Web of Science, and Scopus search revealed 1552 articles, of which 24 were included for the final analysis. 80 patients with autoimmune diseases received CAR-T cell therapy, of which 52 patients had systemic lupus erythematosus, 16 patients had systemic sclerosis, 7 patients had idiopathic inflammatory myopathies, 2 patient had anti-phospholipid antibody syndrome, 2 patients had rheumatoid arthritis, and 1 patient had Sjogren's disease. 44 patients got CD-19 CAR-T and 36 patients got BCMA/CD-19 compound CAR-T. All the patients achieved an immunosuppression-free state at the last follow-up. Of the 47 patients with follow-up data, 79 patients developed cytokine release syndrome (CRS) and 4 patients developed neurotoxicity. None of the patients had fatal adverse events with CAR-T cell therapy. CAR-T appears to be safe and effective in patients with refractory autoimmune diseases. Future studies are crucial to further validate these findings, explore long-term outcomes, and refine the treatment protocols to enhance efficacy and safety.