Rheumatology International最新文献

Limited psychosocial support is available for people with lupus despite the highly reduced quality of life. This study assessed the acceptability, feasibility, and effectiveness estimations, of three (two psychosocial, one exercise) interventions. Lupus patients (N = 124) were randomised to a control arm or one of three interventions delivered remotely over 8-12 weeks: (1) listening support (The Wren project), (2) online Pilates classes, and (3) a text message and video support programme. Online follow up surveys post-intervention and six-months post-baseline included validated instruments for depression (PHQ-8), fatigue (FACIT-F), resilience (CD-RISC), acceptability measures and our co-designed "ADAPT" measure. A subsample of participants completed qualitative interviews. Hedge's g and linear regression were used to estimate effectiveness. All interventions were feasible in terms of recruitment, time, and costs, and met the pre-defined acceptability criteria of > 75% rating the intervention as acceptable/highly acceptable. Helpfulness ratings were highest for listening support with 89% rating it as often/always helpful (62% for Pilates and 52% for Text/videos). Proportions of participants reporting that the intervention had made them feel better mentally often/always was 71% for The Wren, 57% for Pilates and 48% for the text/video group. Qualitatively, the listening support participants valued the "safe space" to talk, and several of the exercise class participants reported improvements to physical and mental health. Although the text message and video programme was acceptable, feasible, and very low cost, 41% of participants would rather have received a different intervention. Suggested text/video adaptations included greater tailoring, particularly to stage of disease journey. Attendance was low for Pilates (only 55% attended > 50% of classes). Estimates of effectiveness favoured all interventions compared to control, although most improvements reduced with time. The interventions were feasible to deliver and acceptable to patients, with indications of potential effectiveness. Further studies are needed to determine effectiveness.Trial registration: ISRCTN72406488.

Anti-carbamylated protein (anti-CarP) antibodies have emerged as novel serologic markers in rheumatoid arthritis (RA). This meta-analysis aimed to assess the diagnostic accuracy of anti-CarP antibodies in RA patients.A systematic Literature search was conducted through April 2025. Primary outcome was diagnostic accuracy of anti-CarP antibodies compared to healthy controls; secondary outcomes included subgroup analyses by anti-citrullinated protein antibodies (ACPA) status.Thirty-six studies (7431 RA patients; 3347 healthy controls) were included. Most used in-house ELISA platforms. Overall, Anti-CarP antibodies showed high specificity but Limited sensitivity in detecting RA with pooled sensitivity and specificity of 44% (95% CI: 39-49%, I²=91.5%) and 96% (95%CI: 94-97%, I²=65.5%), respectively. The pooled diagnostic odds ratio was 14.72 (95%CI: 10.75-20.15, I² = 62.1%), and the area under the summary receiver operating characteristic (SROC) curve was 0.825 (95%CI: 0.797-0.854). In subgroup analysis with studies using carbamylated fetal calf serum as targeted antigen (n = 16), sensitivity and specificity were 41% (95%CI: 38-45%, I²=82.3%) and 96% (95%CI: 95-98%, I²=59.6%). In ACPA-negative patients, sensitivity was 24% (95%CI: 18-31%, I²=87.2%), and specificity was 95% (95%CI: 93-97%, I²=70.3%) with the area under the SROC of 0.755 (95%CI: 0.705-0.805). In ACPA-positive patients, sensitivity was 49% (95% CI: 41-57%, I²=94.0%), and specificity was 95% (95%CI: 93-97%, I²=72.0%) with the area under the SROC of 0.855 (95% CI: 0.806-0.904).Anti-CarP antibodies demonstrate high specificity but limited sensitivity for RA diagnosis. PROSPERO registration: https://www.crd.york.ac.uk/PROSPERO/view/CRD420251065177 .

Takayasu arteritis is a rare inflammatory disease that primarily affects medium- and large-sized arteries, particularly the aorta and its branches. The Hata classification defines six angiographic types based on the involved aortic segments. Clinical symptoms May vary depending on the distribution of arterial involvement. This systematic review and meta-analysis aimed to estimate the pooled prevalence of each angiographic type and evaluate their associations with clinical Manifestations.A systematic search of electronic databases was conducted to identify studies reporting angiographic classifications and clinical symptoms in patients with Takayasu arteritis. Pooled prevalence estimates were calculated using R software, including subgroup analyses by geographic area and imaging modality. Meta-regression was used to assess associations between angiographic types and specific clinical features.Type V was the most common angiographic subtype, with a pooled prevalence of 43.49%, while type III was the least common, 5.32%. Subgroup analyses showed statistically significant differences only for type IIb, based on modality types. Meta-regression revealed significant correlations between angiographic types and clinical symptoms, with Type V exhibiting the greatest severity, and types IIb and III the lowest.This meta-analysis highlights the varying distribution of angiographic types of Takayasu arteritis and their significant associations with clinical symptoms, which may guide prognostic and management strategies.

Mepolizumab (MPZ) is an anti-interleukin-5 monoclonal antibody used to treat eosinophilic granulomatosis with polyangiitis (EGPA). This study aimed to compare the efficacy of MPZ and conventional treatment (CT) for EGPA after maintenance therapy initiation. In this retrospective, observational study, patients diagnosed with EGPA meeting these criteria were included: prednisolone ≤ 20 mg/day, Birmingham Vasculitis Activity Score (BVAS) < 10, and MPZ or new CT initiation ≥ 6 months after initial treatment were included (MPZ: n = 16; CT: n = 16). BVAS, relapse-free survival, absolute eosinophil count, cumulative glucocorticoids (GC) dose, and GC toxicity index (GTI) were evaluated for up to 12 months. Multivariable linear regression for BVAS and logistic regression for relapse at 12 months were performed, adjusting for age, gender, disease duration, and baseline eosinophil count. In the MPZ group, BVAS at 12 months significantly decreased, while BVAS tended to be lower in the MPZ than in the CT group at 12 months. Participants achieving BVAS = 0 significantly increased in the MPZ group at 12 months. Relapse rates tended to be lower in the MPZ group. Absolute eosinophil counts decreased in the MPZ compared with the CT group from 1 to 12 months. Cumulative GC dose and GTI significantly decreased in the MPZ group vs. CT group. In multivariable analyses, the use of MPZ was suggestive of lower BVAS and lower odds of relapse at 12 months compared with CT, although these differences were not statistically significant. MPZ could be a potential treatment option for reducing GC or improving residual symptoms in patients with EGPA.

Systemic sclerosis (SSc) and amyloidosis are rare, complex conditions that impair the function of multiple organs, each with distinct pathogenic mechanisms: autoimmunity for SSc and misfolded protein deposition for amyloidosis. We present the first documented case of a 57-year-old woman with coexisting SSc and systemic AL amyloidosis with multi-organ involvement, in which treatment for amyloidosis led to a notable improvement in SSc symptoms. The patient presented experiencing fatigue, exertional dyspnea, epigastric pain and syncopal episodes in the summer of 2023. Investigations revealed mild increase in left ventricle thickness, elevated NT-proBNP and troponin with negative coronary angiography. She was subsequently diagnosed with SSc with multi-organ involvement and systemic AL amyloidosis confirmed by biopsy. Treatment with a modified Dara-CyBorD protocol led to improvement in SSc symptoms, especially in terms of dyspnea and skin involvement. This is the first reported case of SSc coexisting with systemic AL amyloidosis. The patient responded well to therapy for amyloidosis, suggesting potential overlapping treatment benefits. A multidisciplinary approach was essential, and further studies are needed to explore therapeutic interactions between these two rare diseases.

Despite increasing recognition of axial spondyloarthritis (axSpA) in Latin America, regional data remain scarce. This study aims to describe the clinical, laboratory, and imaging features of axSpA in Latin America using data from the ESPALDA-PANLAR registry. A cross-sectional analysis was conducted using baseline data from the ESPALDA registry, which includes patients with axSpA across seven Latin American countries. Demographic, clinical, laboratory, and imaging data were collected. Patients were stratified by sex, HLA-B27 status, and radiographic classification. Multivariable logistic regression was performed to identify independent associations. A total of 220 patients were included (56% male), with a mean age of 46 years and median diagnostic delay of 93 months (IQR: 13-122). HLA-B27 was positive in 60% of patients, and 62% met radiographic axSpA classification criteria. In multivariable analysis, radiographic axSpA was independently associated with male sex (OR 4.22; 95% CI 1.53-11.59), SI joint erosions on MRI (OR 3.98; 95% CI 1.45-10.90), and longer diagnostic delay (OR 1.01 per month; 95% CI 1.00-1.01). HLA-B27 positivity was associated with bone marrow edema on MRI (OR 2.81; 95% CI 1.50-5.26) and inversely with psoriasis (OR 0.14; 95% CI 0.06-0.33). This first report from the ESPALDA registry highlights distinctive features of axSpA in Latin America, including lower HLA-B27 prevalence and remarkably diagnostic delay. These findings underscore the need for earlier recognition and regionally adapted strategies for diagnosis and management.

Lingual necrosis is a rare but serious complication of giant cell arteritis (GCA). Diagnosis can be difficult due to its atypical presentation, particularly when it occurs without the usual GCA symptoms. We present the case of a 59-year-old female with a history of migraines and smoking, who developed severe tongue pain and neck discomfort. Glucocorticoid therapy was promptly initiated due to clinical suspicion of GCA. However, when the steroid dosage was reduced, the patient's symptoms worsened despite initial improvement. Tocilizumab was subsequently introduced, and by discharge, there was a marked reduction in tongue swelling and evidence of progressive healing. A review of 55 reported cases of lingual necrosis, including ours, revealed an average age of 77.8 years and a female predominance. Hypertension was the most common cardiovascular risk factor, and 32.7% of cases presented with tongue necrosis as the initial manifestation. Most patients had their diagnosis confirmed by temporal artery biopsy, however in other cases, imaging verified the diagnosis. Even though glucocorticoids were still the primary treatment, 13 patients needed other immunosuppressive medications. Tocilizumab has demonstrated promising results in reducing glucocorticoid exposure and improving remission rates. This case highlights the importance of considering GCA in the differential diagnosis of lingual necrosis, even in younger patients or those with atypical presentations. To avoid irreparable consequences, early detection and timely treatment beginning are essential. Tocilizumab may be used as an effective therapeutic option for cases that don't respond to glucocorticoids.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by complex disturbances in both innate and adaptive immune responses, often leading to multi-organ involvement. One of the key features of SLE pathogenesis is endothelial dysfunction, which contributes to immune cell infiltration and vascular inflammation. In this context, adhesion molecules such as platelet endothelial cell adhesion molecule-1 (PECAM-1), intercellular adhesion molecule-1 (ICAM-1), and vascular cell adhesion molecule-1 (VCAM-1) may reflect the degree of endothelial activation. Therefore, we aimed to evaluate serum levels of PECAM-1, ICAM-1, and VCAM-1 in patients with SLE to investigate their potential role as biomarkers of disease activity and future relapse. We investigated 52 patients with SLE: 15 (28.8%) with disease exacerbation (SLE disease activity index [SLEDAI] ≥ 5 points) and 37 (71.2%) in remission (SLEDAI < 5 points), and 12 controls matched by sex and age. All patients met the 2019 EULAR/ACR criteria for SLE. Serum levels of selected adhesion molecules were determined in all participants with the Luminex Discovery Assay Human Premixed Multi-Analyte Kit. We observed no significant differences in the serum levels of PECAM-1 and ICAM-1 between active and inactive SLE patients or between active/inactive SLE patients and healthy controls, but also considering all SLE cases and the control group. However, VCAM-1 levels were 96.8% higher in the active SLE patients (p < 0.001) and 35.4% increase in inactive SLE as compared to controls (p = 0.016), with a similar level between active and inactive SLE patients (p = 0.11). There were no differences in the selected cytokine levels between patients with renal flare and those without renal flare in the active SLE group, but also in inactive SLE group regarding the presence of lupus nephritis despite from 43.3% higher level of ICAM-1 in patients with lupus nephritis (p = 0.016). There were no observed correlations between the levels of these individual cytokines themselves. Furthermore, regarding clinics, only PECAM-1 correlated with disease duration (r_s = 0.42, p = 0.010) and VCAM-1 was associated with SLEDAI (r_s = 0.47, p = 0.003). In the follow-up analysis, during a median observation period of 5.5 years, 10 out of 37 enrolled inactive SLE patients developed a disease flare, but no cytokine differences in baseline levels were found between those with or without a flare in the follow-up period. In our study, VCAM-1 levels were elevated in SLE patients compared to controls, with no significant differences between active and inactive SLE; however, they correlated with laboratory markers of disease activity. PECAM-1 and ICAM-1 showed limited diagnostic utility, though PECAM-1 positively correlated with disease duration.

To identify clinical and demographic predictors associated with the timing of transition from psoriasis (PsO) to psoriatic arthritis (PsA), and to compare the characteristics of patients with concurrent PsO-PsA onset versus those with prolonged transition. A multi-center, observational study was conducted using data from the Turkish League Against Rheumatism (TLAR) network including PsA patients fulfilling CASPAR criteria. Patients were categorized into two groups: Group 1 (concurrent PsO and PsA onset within ± 1 year) and Group 2 (prolonged transition to PsA, > 1 year after PsO). Demographic, clinical, and laboratory characteristics, disease activity, and patient-reported outcomes were compared between groups. Logistic regression was employed to determine independent predictors of prolonged transition. Among 799 patients (mean age 46.8 ± 12.3 years), 237 (29.7%) had concurrent onset and 562 (70.3%) had a prolonged transition, with a mean PsO-to-PsA interval of 12.9 ± 9.6 years. Depression (p = 0.005) and fatigue levels (p = 0.011) were significantly higher in patients with prolonged transition to PsA. Multivariate analysis revealed that scalp psoriasis (OR = 7.162), nail psoriasis (OR = 3.270), family history of PsO (OR = 1.813), and enthesitis ever (OR = 2.187) were associated with prolonged transition. Conversely, family history of PsA (OR = 0.421) and older age at PsO onset (OR = 0.957) predicted shorter transition. Prolonged transition from PsO to PsA is influenced by distinct clinical and demographic factors. Scalp/nail psoriasis, family history of PsO, and enthesitis ever may signal higher risk for prolonged PsA onset. Recognizing these markers can support timely referral and intervention, minimizing diagnostic delay and improving long-term patient outcomes.