BMC Rheumatology最新文献

Objectives: Allogeneic blood transfusion is a common therapeutic intervention for patients with rheumatoid arthritis (RA) undergoing lower extremity joint replacement (LEJR). Despite the potential for adverse outcomes associated with blood transfusion, the risks related to this procedure in RA patients remain underexplored, particularly within the framework of a large-scale national dataset.

Methods: This study analyzed data from the National Inpatient Sample (NIS) database from 2010 to 2019, identifying 66,674 patients diagnosed with RA who underwent LEJR. These cases were subsequently divided into two groups based on whether they received blood transfusion. Univariate and multivariate logistic regression analyses were conducted on patient demographics, the prevalence of comorbidities, hospital-level characteristics, total financial charges, insurance coverage, and in-hospital mortality rates.

Results: The cumulative blood transfusion rate among RA patients undergoing LEJR was 10.9%, showing a declining trend over the study period (from 23.79% in 2010 to 3.67% in 2019). Several factors were associated with an increased likelihood of receiving blood transfusion, including advanced age (≥ 65 years), female sex, deficiency anemia, chronic blood loss anemia, weight loss, coagulopathy, fluid and electrolyte imbalances, neurological disorders, pulmonary circulatory disturbances, congestive heart failure, chronic kidney disease, and uncomplicated diabetes. Moreover, patients who received blood transfusion demonstrated a higher risk of specific complications, including wound infection, acute myocardial infarction, pneumonia, acute kidney injury, urinary tract infection, postoperative delirium, deep vein thrombosis, lower limb nerve injury, sepsis, and respiratory failure.

Conclusion: Thorough preoperative assessment is essential for identifying RA patients who were more likely to receive blood transfusion and be subjected to adverse outcomes. Proactive interventions during the perioperative period, coupled with the implementation of a comprehensive blood management strategy, can optimize blood transfusion in RA patients after LEJR.

Background: Avacopan, a selective oral C5a receptor antagonist, was approved for the treatment of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in 2021. However, there are still limited reports on its efficacy and safety in real-world settings, specifically regarding its impact on the Vasculitis Damage Index (VDI), and its effects on serum biomarkers are poorly understood. This study aimed to evaluate the efficacy and safety of avacopan in remission induction therapy for MPA/GPA in a real-world setting, as well as its effect on serum C5a levels.

Methods: This retrospective study investigated patients with MPA/GPA who received remission induction therapy with a 6-month follow-up at our institution, comparing those who received avacopan with those who did not. Efficacy and safety were evaluated by comparing the remission rate, changes in Birmingham Vasculitis Activity Score (BVAS) and VDI score after 6 months, daily glucocorticoid (GC) dose, and incidence of adverse events (AEs). Changes in serum C5a levels, measured using ELISA, were compared between both groups at baseline and 3 months.

Results: A total of 66 patients with MPA/GPA were included, with 14 and 52 patients in the avacopan and non-avacopan groups, respectively. The remission rate and decrease in BVAS was comparable between both groups. However, those who received avacopan had a significantly smaller increase in VDI score, significantly lower daily GC dose at 1, 3, and 6 months, and significantly lower incidence of GC-related AEs within 6 months. Serum C5a levels did not significantly change in the avacopan group but significantly decreased in the non-avacopan group. Remission was achieved in the avacopan group regardless of whether serum C5a decreased or increased.

Conclusions: Treatment with avacopan appears to effectively suppress the increase in VDI score, enable reduced GC dosage, and lower the incidence of GC-related AEs during remission induction therapy for MPA/GPA in a real-world setting. Furthermore, avacopan may suppress disease activity regardless of serum C5a levels.

Clinical trial number: Not applicable.

Background: This study was conducted to describe differences in self-reported health-related quality of life (HRQoL) for patients with inflammatory joint disease (IJD) related to sociodemographic factors.

Methods: The data were collected through an anonymous survey in a cross-sectional study of 261 patients with IJD- rheumatoid arthritis (RA), psoriatic arthritis (PsA) and axial spondyloarthritis (axSpA). The patients' health status was assessed using a standard questionnaire [EQ-5D-3L].

Results: The results showed no significant differences related to the type of JDC in any domain of patients' quality of life. Among the demographic factors, social status showed a significant association with all aspects of patients' HRQoL: mobility ratings (p = 0.002), self-care ratings (p < 0.001), usual activities (p < 0.001), pain or discomfort (p = 0.039), anxiety or depression (p = 0.001). Anxiety and depression were more common among women than men (p = 0.033). Men rated their health higher on the EQ-VAS scale (p = 0.036). Working patients reported better health than retirees (p = 0.008), and disability pensioners (p < 0.001). Better health was associated with higher levels of education (p < 0.001). Patients with elementary education provided the lowest ratings while patients with higher degrees gave the highest ratings. Patients living in villages reported better health than those from urban areas (p = 0.019). Social class, education, and place of residence accounted for 17.9% of the variance in EQ-VAS scores.

Conclusion: Understanding the role of sociodemographic factors is crucial to promote improved patient care and better healthcare resources. The results of our study can serve as a benchmark for future studies to assess the influence of sociodemographic factors among patients with other subtypes of IJD.

Introduction: Rheumatoid arthritis (RA) in adult patients, there is contradictory evidence regarding Abatacept's safety profile (ABA). This study aims to assess the safety and efficacy of ABA in adult patients in Saudi Arabia.

Methods: This retrospective cohort study analyzed adult patients aged 18 and above with RA who received ABA at King Fahad Armed Forces Hospital, and King Fahad General Hospital, and Al-Noor Specialist Hospital in Saudi Arabia. Data was collected from electronic medical records, and analyzed using the statistical analyses (IBM's SPSS Software, version 29.0).

Results: The study included 236 RA patients (88.6% female), with a mean age of 55.7 years. Comorbidities were present in 64.6%, and the average disease duration was 127.1 months. Joint erosion was the most common feature (49.6%), while 25% had extra-articular manifestations. Abnormal labs included elevated liver enzymes and leukocytosis. After 6 months of ABA, DAS-28 scores significantly decreased to a mean of 3.07 (SD = 1.31; p < 0.001). The mean treatment duration was 28.0 months, with a 31.8% discontinuation rate-mainly due to secondary failure (41.1%), primary failure (17.9%), and non-compliance (10.7%). Discontinuation was more frequent in females (p = 0.049). ADRs (Adverse drug reactions) included cytopenia in 8.6% (n = 18), mainly anemia. liver enzyme elevations, GI upset, HBV reactivation, and one malignancy, but none were statistically significant (all p > 0.05). tuberculosis (TB) reactivation occurred in 2 patients (0.8%), neither discontinued the drug (p = 0.565). Notably, 45.3% were biologic-naïve and showed better outcomes: greater DAS-28 reduction (2.1 vs. 1.5; p = 0.015) and lower discontinuation rates (24.3% vs. 38.8%; p = 0.028) than biologic-switch patients.

Conclusion: The study confirms the safety and efficacy of ABA in treating RA in Saudi Arabian adults. It found significant improvements in disease activity, but with high discontinuation rates though aligning with previous studies. We recommend measures targeting identified possible causes and extensive research to explore the safety and efficacy of ABA.

Clinical trial number: Not applicable.

Background: Immune checkpoint inhibitors (ICIs) have become a cornerstone in the treatment of metastatic melanoma. Several case reports have documented IgG4-related disease (IgG4-RD) as an adverse event following ICI therapy. Here we report the first instance of interstitial nephritis associated with IgG4-RD as an immune-related adverse event (irAE) following ICI treatment.

Case presentation: A 71-year-old male with malignant melanoma (BRAF wild-type) initially received one cycle of adjuvant pembrolizumab, followed by four cycles of ipilimumab/nivolumab after the occurrence of lung metastases. Four months later, a follow-up computed tomography (CT) revealed infiltrative masses in the kidneys, along with abnormal mediastinal and hilar lymphadenopathy but his baseline serum creatinine remained stable. A subsequent kidney biopsy showed renal parenchyma with significant interstitial nephritis and an increase in IgG4-positive plasma cells, with no evidence of malignancy. Plasma IgG4 levels were elevated at 294 mg/dL (normal 11-157 mg/dL), and complement C4 level was low at < 8 mg/dL. In addition, the patient had an asymptomatic rise in lipase (105 U/L, normal 7-60 U/L), but had no other findings to suggest pancreatitis. The patient was started on prednisone 40 mg daily with a plan to taper. A follow-up CT scan performed four weeks later showed near-complete resolution of the previously observed mediastinal lymphadenopathy and bilateral infiltrative renal masses.

Conclusion: This represents the first reported case of interstitial nephritis resulting from IgG4-related disease following ICI treatment. Clinicians should consider the potential for IgG4-RD, particularly with associated renal manifestations, in patients undergoing ICI therapy. Early recognition and treatment of this rare side effect can significantly impact the clinical outcome. This case highlights the importance of being vigilant for uncommon and new adverse effects following ICI treatment, especially as the field continues to evolve and new immunotherapies are developed.

Clinical trial number: Not applicable.

Background: Idiopathic inflammatory myopathies (IIM) are a group of related chronic autoimmune diseases characterized by muscle inflammation and numerous other potential organ specific manifestations. People with IIM often present with reduced muscle strength, endurance, and aerobic capacity, directly impacting physical function and health-related quality of life. With emerging evidence supporting exercise in IIM, we sought to explore the experiences of exercise in people with IIM to further inform person-centered exercise interventions.

Methods: Semi-structured interviews were conducted with IIM patients attending the rheumatology clinic at Royal Prince Alfred Hospital, Sydney, Australia. Interviews were audio-recorded, transcribed, de-identified using alphanumeric codes, and analyzed thematically.

Results: Twenty adults (women = 12, men = 8) with a mean age of 52.6 ± 12.9 years and a mean disease duration of 9.5 ± 8.9 years were included. Nine themes emerged: barriers to exercise (5 themes) and facilitators to exercise (4 themes). Barriers to exercise include (1) variability of disease burden (day-to-day symptom fluctuation, episodic flares, and side effects of treatments), (2) spectrum of disease severity, (3) fear of disease exacerbation, (4) navigating logistical conflict and (5) exercise and disease knowledge deficiency (lack of exercise knowledge in people with IIM and lack of disease-specific knowledge in exercise providers). Facilitators to exercise include (6) knowledge empowerment (participant education on the benefits of exercise in IIM to empower exercise engagement, and disease-specific education for exercise providers to facilitate understanding and trust with their patients) (7) improving exercise motivation through physical and mental wellbeing, (8) promoting positive affect to improve exercise engagement and adherence (social involvement and distractions), and (9) individualizing exercise to clinical and social circumstances.

Conclusions: People with IIM experience several barriers to exercise including disease severity, symptom unpredictability, fear of disease exacerbation, and difficulty scheduling exercise around medical appointments and life commitments. Education about the role of exercise and individualising exercise for people with IIM are central to improving exercise engagement and confidence. It is also important for health care providers to support people with IIM in making the link between physical and mental well-being and maintenance of independence and quality of life.

Background: Behçet's Disease (BD) is a chronic, systemic vasculitis of unknown etiology that affects multiple organ systems. It is characterized by recurrent oral and genital ulcers, ocular involvement, affecting arteries and veins of all sizes. It is more prevalent in countries along the ancient Silk Road. Diagnosis is primarily clinical, as there are no specific laboratory tests. The International Criteria for BD (ICBD) was developed to improve diagnostic accuracy. Management requires a multidisciplinary approach, with treatment strategies depending on disease severity. Despite BD's significant morbidity and diverse clinical manifestations, its prevalence and characteristics remain to be described in Palestine. This research provides critical insights into disease patterns and contributes to improved diagnosis and management in Palestine.

Methods: A retrospective cohort study was conducted in the period from Aug 2024 until March 2025 in rheumatology clinics across the West Bank and Jerusalem. 60 Patients diagnosed with BD based on ICBD (score ≥ 4) were included. Exclusion criteria were cognitive impairment, incomplete records, or non-residence in Palestine. Sixty patients were enrolled. Data was collected via chart review and patient interviews. Disease-related complications were assessed using the Behçet's Overall Damage Index (BODI) to ensure standardized evaluation.

Results: The male to female ratio was 1.14:1. In addition, the most common initial clinical presentations were oral aphthous ulcers (96.7%), genital aphthous (86.7%), ocular lesions (66.7%), skin lesions noted (46.7%), while vascular lesions occurred in (30%). Neurological manifestations and positive Pathergy test were (25%) and (18.3%), respectively. Regarding complications, the most common were vascular events (36.7%), skin ulcerations (33.3%), mucocutaneous scars (20%), avascular necrosis (13.3%), osteoporotic fractures (10%). Regarding complications in the eye, anterior segment changes presented (15%), posterior segment changes (8.3%), visual impairment in one eye (33.3%), and (13.3%) in both. Neurological complications were less frequent and there was no difference in characteristics between the genders.

Conclusion: The most common manifestations were oral aphthous ulcers, followed by genital ulcers, neurological manifestations, and pathergy was the least frequent. The most frequently reported complications were vascular events, skin ulceration, and visual impairment, while neuropsychiatric complications were the least frequent, and there was no gender difference in BD characteristics.

Research registry number: No trial registry number.