The COVID-19 Pandemic Heightens Interest in Cytokine Storm Disease and Advances in Machine Learning Diagnosis, Telemedicine, and Primordial Prevention of Rheumatic Diseases.

Insights gained during the coronavirus disease 2019 pandemic has underscored the critical role played by both innate and adaptive immune responses in determining the severity of diseases. This newfound understanding holds significant potential to bring about a paradigm shift in the diagnosis, treatment, and management of autoimmune conditions. Advanced technologies that are emerging in the field are expected to play a pivotal role in this transformation.

Recent Advances in Pathogenesis, Diagnostic Imaging, and Treatment of Sjögren's Syndrome.

It is our pleasure to present the Special Issue "Diagnosis and Treatment of Sjögren's Syndrome" to the readers of the [...

Preferable effect of CTLA4-Ig on both bone erosion and bone microarchitecture in rheumatoid arthritis revealed by HR-pQCT.

This exploratory study aimed to examine the impact of abatacept treatment on bone structure in patients with rheumatoid arthritis (RA) using high-resolution peripheral quantitative computed tomography (HR-pQCT). RA patients initiating either abatacept or newly introduced csDMARDs were enrolled in this prospective, non-randomized, two-group study. Bone structure in the 2nd and 3rd metacarpal heads was assessed using HR-pQCT at 0, 6, and 12 months after enrollment.

Investigating the impact of tocilizumab on serum cytokines concentrations in Japanese FMF patients: a sub-analysis of the NUH01FMF study.

Familial Mediterranean Fever (FMF) is the most common hereditary autoinflammatory disease, characterized by recurrent fever, arthritis, rash, and serositis, and is caused by mutations in the MEFV gene coding for the pyrin protein. The primary treatment goal is to prevent acute attacks and minimize subclinical inflammation to avoid secondary amyloidosis with colchicine as the first-line treatment. However, 10-20% of patients are colchicine-resistant or intolerant.

Essential role of interferon-regulatory factor 4 in regulating diabetogenic CD4+ T and innate immune cells in autoimmune diabetes in NOD mice.

Haploinsufficiency of the transcription factor interferon-regulatory factor 4 (IRF4) prevents the onset of spontaneous diabetes in NOD mice. However, the immunological mechanisms of the IRF4-mediated disease regulation remain unclear. This study aims to investigate the role of IRF4 in the pathogenesis of autoimmune diabetes by conducting adoptive transfer experiments using donor IRF4 gene-deficient CD4+ T cells from BDC2.

Effectiveness and Safety of Avacopan as a Combination Therapy with Glucocorticoids or Monotherapy in Patients with Microscopic Polyangiitis.

We herein report three cases of microscopic polyangiitis (MPA). Two patients were administered avacopan in combination with glucocorticoid (GC), whereas one patient was treated with avacopan monotherapy; none of the patients were co-administered either rituximab or cyclophosphamide. The doses of GC were successfully reduced after the introduction of avacopan in the two patients, and the serum C-reactive protein levels decreased in the patient treated with avacopan monotherapy.

Microarchitectural analysis of the metacarpophalangeal joint using HR-pQCT in patients with rheumatoid arthritis: A comparison with healthy controls.

Objective: To investigate which joint microarchitectural parameters measured by high-resolution peripheral quantitative computed tomography (HR-pQCT) serve as imaging markers for rheumatoid arthritis (RA).

Methods: The second and third metacarpophalangeal (MCP) joints of 50 patients with RA and 50 healthy controls (HCs) (aged 50-79 years, all females) were scanned using a HR-pQCT. Joint space, trabecular bone microarchitecture, and erosion were measured and compared between RA patients and HCs.

[Open Surgery for Right Subclavian Artery Aneurysm with Infective Endocarditis:Report of a Case].

Subclavian artery aneurysm is usually rare. We report a case of a right subclavian artery aneurysm with infective endocarditis. A 36-year-old woman was admitted at our hospital due to a cerebral embolism.

A Randomized Controlled Trial on the Effect of Luseogliflozin on Bone Microarchitecture Evaluated Using HR-pQCT in Elderly Type 2 Diabetes.

Introduction: Bone fragility is a critical issue in the treatment of elderly people with type 2 diabetes (T2D). In the Canagliflozin Cardiovascular Assessment Study, the subjects with T2D who were treated with canagliflozin showed a significant increase in fracture events compared to a placebo group as early as 12 weeks post-initiation. In addition, it has been unclear whether sodium-glucose co-transporter 2 (SGLT2) inhibitors promote bone fragility.

Exercise-associated Hyponatremia Developing Immediately after a Musical Stage Performance in a Healthy Actress.

Exercise-associated hyponatremia (EAH) is a life-threatening dilutional hyponatremia that typically occurs during or immediately after exercise in endurance athletes. A 49-year-old actress experienced dizziness 15 min after a 2-h stage performance while drinking several bottles of water. Thirty minutes later, the patient fell unconscious and was hospitalized.

Biomarkers and Signaling Pathways Implicated in the Pathogenesis of Idiopathic Multicentric Castleman Disease/Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly (TAFRO) Syndrome.

Idiopathic multicentric Castleman disease (iMCD) and TAFRO syndrome present a variety of symptoms thought to be caused by excessive inflammatory cytokines and chemokines, but the underlying mechanisms are unknown. iMCD is broadly classified into two types: iMCD-NOS and iMCD-TAFRO, which have distinct laboratory findings, pathological features, and responses to treatments. It is thought that iMCD-NOS, particularly the IPL type, responds favorably to IL-6 inhibitors due to its IL-6-centric profile.

Influences of advanced age in rheumatoid arthritis: A multicentre ultrasonography cohort study.

Objectives: We aimed to evaluate the effects of age on clinical characteristics and outcomes in biologic or targeted synthetic disease-modifying antirheumatic drug (b/tsDMARD)-naïve patients with rheumatoid arthritis (RA).

Methods: We analysed the cases of 234 Japanese b/tsDMARD-naïve RA patients who underwent b/tsDMARD treatment in a multicentre ultrasound prospective observational cohort. We compared the clinical characteristics at baseline and outcomes at 12 months between those aged ≥60 years and those <60 years.

Period prevalence of uveitis in human T-lymphotropic virus 1 carriers versus noncarriers in a highly endemic area: The Nagasaki Islands Study.

The magnitude of the effect of human T-lymphotropic virus 1 (HTLV-1) infection on uveitis remains unclear. We conducted a cross-sectional study in a highly endemic area of HTLV-1 in Japan. The study included 4265 residents (men, 39.

Fatal Systemic Capillary Leak Syndrome in a Patient with a COVID-19 Infection.

A 29-year-old Japanese woman was admitted to our hospital with a fever, cardiogenic shock, and cardiac arrest. Laboratory data indicated multiple organ failure in addition to hemoconcentration, hypoalbuminemia, and myocardial damage. The coronary angiography findings were normal, and fulminant myocarditis was suspected.

TERT Promoter Mutations Increase Tumor Aggressiveness by Altering TERT mRNA Splicing in Papillary Thyroid Carcinoma.

Context: Telomerase reverse transcriptase promoter (TERT-p) mutations, which upregulate TERT expression, are strongly associated with tumor aggressiveness and worse prognosis in papillary thyroid carcinomas (PTCs). TERT expression is also observed in a proportion of PTCs without TERT-p mutations, but such tumors show less aggressiveness and better prognosis than TERT-p mutation-positive tumors.

Objective: TERT has multiple splicing variants whose relationships with the TERT-p status and clinicopathological characteristics remain poorly understood.