全身性エリテマトーデス

In systemic lupus erythematosus (SLE) and other autoimmune diseases, B-cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL) play critical roles through three receptors, i.e., BAFF-Receptor (BAFF-R), transmembrane activator and calcium-modulator and cyclophilin ligand (CAML) interactor (TACI), and B-cell maturation antigen (BCMA), promoting the survival of self-reactive B cells and supporting their differentiation into antibody-producing cells. Several biologic therapies with anti-BAFF and anti-BAFF/APRIL agents induce profound suppression of immunoglobulin production and B-cell survival with variable safety profiles. While belimumab has demonstrated significant improvement of overall disease activity in clinical trials, some cases of de novo lupus nephritis have been reported suggesting a potential lack of protection in certain cases. The sustained reduction in B cell populations expressing regulatory markers, alongside a rapid decline in IL-10 levels following belimumab initiation, suggests that BAFF may have a previously underappreciated role in supporting the development or function of regulatory B cells (Bregs). Given the complex receptor interactions of BAFF and APRIL, as well as the heterogeneous phenotype of regulatory B cells and their different mechanisms for regulating the immune response, the involvement of BAFF in regulatory immune responses remains difficult to fully elucidate. This review explores the current evidence on the anti-BAFF/APRIL therapies and examines the unresolved question of whether BAFF exerts a context-dependent role in supporting regulatory B cell function.

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by autoantibody production and multi-organ involvement due to dysregulated immune responses. Ongoing research aims to understand the underlying mechanisms of this immune dysregulation, with particular interest in immune checkpoint molecules such as VISTA (encoded by the VSIR gene), which exerts context-dependent immunomodulatory effects. We analyzed a large-scale single-cell RNA-seq (scRNA-seq) dataset (GSE174188) comprising 261 samples (162 SLE cases, 99 controls) to investigate VSIR expression patterns across peripheral blood mononuclear cell (PBMC) subsets in SLE versus controls, using bioinformatics tools. Additionally, fresh PBMCs were isolated from SLE patients and healthy controls, and VSIR mRNA levels were quantified by RT-qPCR in treatment-naïve cases and those receiving Prednisolone, Hydroxychloroquine, or supplementary medications. Associations between VSIR expression and clinical/demographic parameters, including disease activity index, were also evaluated. scRNA-seq analysis revealed significantly upregulated VSIR expression in monocytes from SLE patients compared to healthy controls. In contrast, pseudobulk differential expression analysis of the entire PBMC population, corroborated by RT-qPCR on fresh samples, demonstrated downregulation of VSIR in SLE cases overall (log₂ fold change = -1.23). Notably, VSIR expression differed across treatment groups, with the lowest levels observed in treatment-naïve patients and higher levels in those receiving first-line therapies (prednisolone and hydroxychloroquine). Furthermore, VSIR expression exhibited a significant negative correlation with SLE disease activity index. This study demonstrates decreased VSIR expression in the whole PBMC population in SLE patients, suggesting a potential role in disease pathogenesis processes possibly through altered immune checkpoint regulation. Moreover, VSIR expression was associated with treatment status, with higher expression observed in patients receiving standard first-line therapies.

Kikuchi-Fujimoto disease is a rare, self-limiting autoimmune necrotizing lymphadenitis that poses unique diagnostic challenges due to vague, overlapping clinical and laboratory features with numerous important and common differential diagnoses. Accurate diagnosis is needed to avoid unnecessary and unwanted treatment. Herein, we report a unique case of a middle-aged diabetic man from South India presenting with prolonged fever, significant weight loss, and generalized lymphadenopathy. Laboratory findings revealed leukopenia, markedly elevated inflammatory markers, and a moderately positive antinuclear antibody titer, raising immediate concern for systemic lupus erythematosus. Results of an extensive infectious workup, including tuberculosis testing, were negative. The definitive diagnosis was established by lymph node biopsy demonstrating necrotizing lymphadenitis with karyorrhectic debris and crescentic histiocytes, without caseating granulomas or hematoxylin bodies. This case underscores the need for early lymph node biopsy in tuberculosis-endemic settings to avoid misdiagnosis and inappropriate antitubercular therapy and also highlights the emerging association between Kikuchi-Fujimoto disease and autoimmune serological markers.

BACKGROUND/AIMS: Growing evidence indicates that gut microbiota dysregulation plays a crucial role in the pathogenesis of systemic lupus erythematosus (SLE); yet, a quantitative overview of research activity in this field is lacking. This study aimed to provide a bibliometric analysis of global research trends and thematic evolution concerning gut microbiota and SLE between 2014 and 2025. MATERIALS AND METHODS: Relevant publications were retrieved from the Web of Science Core Collection and Scopus databases, and only original research articles and review articles published in English between January 1, 2014, and December 31, 2025, were included. Bibliometric analyses were performed using the bibliometrix package in R and CiteSpace to evaluate publication trends, country and institutional contributions, collaboration networks, keyword co-occurrence, and citation burst patterns. RESULTS: A total of 525 publications were included, comprising 248 original articles and 277 reviews. Annual publication output increased steadily, with accelerated growth after 2019 (annual growth rate of 121.20%), and 91 papers were published in 2025. China and the United States were the main contributors, accounting for more than half of the total publications, with field-normalized contributions of 1.0927 and 1.2268. The Medical University of South Carolina and Zhejiang Chinese Medical University were the most productive institutions (10 articles). Citation burst analysis showed that "Intestinal dysbiosis associated with systemic lupus erythematosus" laid the foundation for research in this field. Keyword analysis indicated a gradual shift from descriptive microbial characterization toward themes related to immune regulation and dysbiosis. In addition, increasing research attention has been directed toward microbiota-based therapeutic approaches in SLE. CONCLUSION: Research on gut microbiota in SLE has expanded substantially over the past decade, with an observable shift from association-based studies toward more mechanistic and translational research themes. Distinct regional research emphases were noted, highlighting the potential value of enhanced international and interdisciplinary collaboration in advancing research in this field. Cite this article as: Yang J, Wang J. Research trends and emerging themes in gut microbiota-systemic lupus erythematosus research: A bibliometric analysis (2014-2025). ArchRheumatol. 2026;41(3):249-257.

Objective: To explore the clinical characteristics and prognosis of patients with thyroid cancer complicated by connective tissue disease. Methods: A retrospective analysis was conducted on the clinical data of 70 patients with thyroid cancer complicated by connective tissue disease who were admitted to the Cancer Hospital, Chinese Academy of Medical Sciences from May 30, 2006, to April 30, 2024 and telephone follow-up was performed. Results: Among the 70 patients with thyroid cancer complicated by connective tissue disease, 68 cases (97.1%, 68/70) had papillary thyroid carcinoma, and 2 (2.9%, 2/70) had medullary thyroid carcinoma; 67 cases (95.7%, 67/70) were female. The age at diagnosis of thyroid cancer was (48.73±11.74) years, and the median interval from connective tissue disease diagnosis to thyroid cancer diagnosis was 7 years (range, 3 to 12 years). Among them, there were 29 cases (41.4%, 29/70) of rheumatoid arthritis, 24 cases (34.3%, 24/70) of Sjögren's syndrome, 12 cases (17.1%, 12/70) of systemic lupus erythematosus, 3 cases (4.3%, 3/70) of systemic sclerosis, and 2 cases (2.9%, 2/70) of dermatomyositis. The stages of thyroid cancer were stage Ⅰ in 55 cases (78.6%, 55/70), stage Ⅱ in 11 cases (15.7%, 11/70), stage Ⅲ in 1 case (1.4%, 1/70), and unknown in 3 cases (4.3%, 3/70). Multifocality was observed in 37 cases (52.9%,37/70), capsular invasion in 46 (65.7%,46/70) cases, perineural invasion in 1 (1.4%,1/70) case, and lymphovascular invasion in 3 (4.3%,3/70) cases. The median follow-up time was 73.50 months (range, 20.9 to 235.0 months). Six patients were lost to follow-up, one patient died of COVID-19 and the remaining 63 patients (98.4%, 63/64) did not experience recurrence or metastasis. Conclusions: Patients with thyroid cancer complicated by connective tissue disease are mostly female, and the predominant pathological type is papillary thyroid carcinoma. Most patients are diagnosed at an early stage. No recurrence or metastasis was observed during follow-up,and further confirmation by large-sample studies is needed.