Archives of rheumatology最新文献

Behçet's syndrome is a variable vessel vasculitis characterized by a diverse range of clinical manifestations resulting from inflammation involving several organs and systems. While significant progress has been made in understanding the pathogenesis and treatment of Behçet's syndrome, challenges remain in achieving optimal disease control and preventing long-term complications. This review explores recent advances in the management of Behçet's syndrome, with a focus on emerging therapies and future directions. Apremilast, a phosphodiesterase-4 inhibitor, has shown promise in managing mucocutaneous manifestations, particularly oral ulcers. Tocilizumab, an interleukin (IL)-6 receptor inhibitor, has demonstrated efficacy in certain patient populations, especially those with ocular involvement. However, its use in vascular Behçet's syndrome requires careful consideration. Relapses of oral and genital ulcers can be challenging during tocilizumab treatment. Other emerging therapies, such as IL-17 inhibitors, including secukinumab and ixekizumab, IL-12/23 inhibitor ustekinumab, and Janus kinase (JAK) inhibitors, including tofacitinib and baricitinib, are being investigated for their potential to target specific inflammatory pathways. Future research directions include the development of novel therapeutic targets, better use of existing agents by identifying patient populations that would benefit from these, developing better instruments for disease assessment, and a treat-to-target approach in order to improve outcomes and quality of life for patients with Behçet's syndrome.

Objectives: This study aims to explore the value of ultrasonic semi-quantitative scoring in the diagnosis of seronegative rheumatoid arthritis (RA).

Patients and methods: Between January 2018 and October 2023, a total of 411 patients (241 males, 170 females; mean age: 50.9±17.5 years; range, 18 to 87 years) were included. Of these patients, 296 were diagnosed with RA (including 131 with seronegative RA [SNRA] and 165 with seropositive RA [SPRA]) and 115 with non-RA disease. Ultrasound examination was performed on all patients with suspected RA, focusing on evaluation of synovial hypertrophy (SH), power Doppler (PD) signals, and bone erosion (BE) for three to six months. The ultrasonic joint semi-quantitative score was evaluated for the sensitivity and specificity of detecting seronegative RA.

Results: The three indexes of SH, PD, and BE were not significantly different between the SNRA and SPRA groups (p=0.223, p=0.176; p=0.272, respectively). However, there were differences on the SH1, SH3, PD, and BE grades between the SNRA group and the non-RA group (p<0.001 for all); when serology was negative and when the highest scored joint met PD Grade ≥2 or BE Grade ≥2, it showed both high sensitivity (93.12%) and high specificity (91.30%) for the diagnosis of RA.

Conclusion: Ultrasound combined with semi-quantitative scoring is of promising significance in the early diagnosis of SNRA patients.

Objectives: This study aimed to determine the frequency of autoimmune diseases (ADs) accompanying common variable immunodeficiency (CVID) and evaluate clinical and immunological features, organ manifestation, and effects on malignancy and mortality.

Patients and methods: The retrospective study was conducted with 85 patients (47 males, 38 females; median age: 38 years; range, 30 to 53 years) with CVID between January 2013 and January 2023. The patients were divided into two groups according to the presence of ADs: CVID patients with ADs [AD-CVID (+) group; n=36] and CVID patients without ADs [AD-CVID (-) group; n=49]. The clinical and immunological features of the groups were compared, and the effects on organ manifestations, malignancy development, and mortality were evaluated.

Results: The diagnostic delay in the AD-CVID (+) group was 84 months and was longer than that in the AD-CVID (-) group. The most common AD was cytopenia, particularly immune thrombocytopenic purpura. Splenomegaly was the most common organ manifestation. Sjögren syndrome was the most common rheumatic disease. There was no difference between the immunoglobulin levels and lymphocyte subgroup levels, whereas the class-switched memory B cell levels were lower in the AD-CVID (+) group. While malignancy, particularly non-Hodgkin lymphoma, was more common in the AD-CVID (+) group, no difference was observed in mortality between the groups.

Conclusion: Adult CVID patients with ADs have a longer diagnostic delay. Autoimmune conditions, particularly autoimmune cytopenias and inflammatory diseases, are much more common in patients with CVID than in the general population. Therefore, physicians' awareness of autoimmune manifestations in CVID patients should be increased to prevent delays in diagnosis.

Objectives: The purpose of this study was to evaluate Eustachian tube function in patients with Behçet's disease (BD).

Patients and methods: Forty-six patients (22 males, 24 females; mean age: 42.1±11.3 years; range, 19 to 64 years) with BD and 46 (21 males, 25 females; mean age: 38.5±14.8 years; range, 19 to 63 years) age- and sex-matched audiologically healthy individuals were enrolled in this cross-sectional, case-control study between June 2023 and August 2023. Demographic and clinical characteristics of participants were recorded from electronic health records. All participants completed the Eustachian tube function test and the Eustachian tube dysfunction questionnaire 7 (ETDQ-7). Binary logistic regression analysis was employed to identify the factors that predict Eustachian tube dysfunction in patients with BD. Additionally, the association between disease duration and ETDQ-7 scores was evaluated using Spearman's rank correlation.

Results: The median ETDQ-7 scores were significantly higher in patients with BD than in controls (10 (7-32) vs. 7 (7-9); p<0.001). The number of ears with Eustachian tube dysfunction was also significantly higher in the BD group than in the controls (n=22, 47.8% vs. n=7, 15.2%; p=0.007). Regression analysis did not reveal statistically significant factors that predicted Eustachian tube dysfunction. Moreover, no statistically significant correlation was observed between Eustachian tube dysfunction and disease duration (R=-0.067; p=0.525).

Conclusion: This study showed that Eustachian tube dysfunction is more prevalent in patients with BD than in the healthy controls. However, clinical and demographic variables were not found to be associated with Eustachian tube dysfunction.

Objectives: This study aimed to evaluate the performance of the Behçet's Syndrome Overall Damage Index (BODI) in an adult Egyptian cohort.

Patients and methods: This longitudinal retrospective cohort study included 282 adult patients (233 males, 49 females; mean age: 35.3±8.7 years; range, 16 to 66 years) with Behçet's disease (BD) between January 1980 and December 2022. BODI was assessed regarding construct validity, sensitivity to change, and intra- and inter-rater reliability. The ability of BODI to discriminate between activity and damage was evaluated. Its performance in another vasculitis syndrome was assessed in 12 patients with antineutrophil cytoplasmic antibody-associated vasculitis.

Results: BODI captured more damage items compared to the Vasculitis Damage Index (VDI). BODI scores were not correlated with disease activity and had poor performance in other vasculitides. BODI had a trend of progressive increment over time. It showed consistence when reassessed by the same rater and by different raters. Some damage items in the study cohort were lacking in BODI.

Conclusion: BODI is more comprehensive compared to VDI. It shows good face, construct, and discriminant validity. It is sensitive to change and has good intra- and inter-rater reliability. Newer versions of BODI are recommended to increase score comprehensiveness.

Objectives: This study aims to investigate the thermal characteristics of the small joints of the hands between patients with rheumatoid arthritis (RA) and healthy controls.

Patients and methods: Between December 2020 and May 2021, a total of 52 RA patients (9 males, 43 females; mean age: 52.1±11.1 years; range, 38 to 68 years) who met the revised American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and 26 healthy controls (10 males, 16 females; mean age: 51.2±8.2 years; range, 38 to 68 years) were included. Joint tenderness was evaluated using Ritchie articular index (RAI). Joint tenderness was scored from 0 to 3. Thermal data were collected from the hand regions of individuals. A FLIR T450sc microbolometer infrared thermal camera with 320×240 resolution was used for the thermography of individuals. Bilaterally proximal interphalangeal joints (1-5) and metacarpophalangeal joints (1-5) were evaluated. The mean temperature was compared between the patients and healthy controls.

Results: The mean disease duration of patients with RA was 10.4±8.9 years. The mean temperature values of the joints in the patients with a RA RAI score of 0, 1, 2, 3 were 32.43±1.59°C; 32.71±1.36°C; 33.12±1.23°C; 33.60±0.99°C, respectively. The mean temperature was 31.14±1.51°C in healthy controls. The mean temperature values of the joints in the RA patients with RAI score of 0 was higher compared to healthy controls (p<0.05). Patients with a Ritchie sensitivity score of 1 had a higher mean temperature compared to patients with score of 0 (p<0.05). In RA patients, the joints with a RAI score of 1 had higher mean temperature values than the joints with RAI score of 0 (p<0.05). The mean temperature values of the joints with RAI score of 2 were also higher than the joints with RAI score of 1 (p<0.05).

Conclusion: Our study results suggest that thermal imaging may be an objective tool for diagnosis and assessing disease activity in RA.

Objectives: The study aimed to investigate the possible effects of coronavirus disease 2019 (COVID-19) on autoantibodies.

Patients and methods: Samples of 89,108 individuals (29,033 males, 60,075 females; median: 36 years; range, 0 to 96 years) who underwent autoimmune testing between January 2017 and May 2022 were retrospectively analyzed. The prepandemic period was defined as May 1, 2017, to March 20, 2020, while the pandemic period was defined as March 20, 2020, to May 31, 2022.

Results: Of the participants, 0.55% were of foreign nationality. The positivity rate was 18.12%. Autoantibody positivity rates, when analyzed by sex, were higher in females for antinuclear antibody (ANA), antimitochondrial antibody (AMA), anti-liver kidney microsomal (LKM) antibody, immunoglobulin A (IgA) anti-gliadin antibody, anti-endomysial antibody A, anti-ribosomal P protein antibody, anti-Sjögren's syndrome A (anti-SSA), anti-Sjögren's syndrome B (anti-SSB), anti-Smith/ribonucleoprotein (anti-SM/RNP), anti-SM, and c-ANCA (cytoplasmic antineutrophil cytoplasmic antibody). When the prepandemic period was compared with the pandemic period, AMA, anti-LKM antibody, IgA anti-gliadin antibody, anti-endomysial antibody A, and anti-SM/RNP levels were higher in the prepandemic period, while ANA was higher during the pandemic. Additionally, statistically significant differences were found in the distributions of ANA, AMA, anti-LKM antibody, IgA anti-gliadin antibody, anti-endomysial antibody A, anti-ribosomal P protein antibody, anti-SM, anti-SSA, and c-ANCA across the years.

Conclusion: This study could not establish a cause-effect relationship between the changing autoantibody levels during the COVID-19 pandemic and severe acute respiratory syndrome coronavirus 2 infection due to the lack of results from the same patients across different periods. Nonetheless, we believe the quantitative seroprevalence changes in such a large sample of autoantibody screening results over a five-year period, including the pandemic, are valuable.