Etiopathogenesis of Behçet's disease: A systematic literature review.

Behçet's disease (BD) is a chronic, multisystemic inflammatory vasculitis affecting veins and arteries. Its etiopathogenesis remains unclear but is thought to result from genetic predisposition combined with environmental triggers. Recent genome-wide association studies (GWAS) have linked various genetic polymorphisms (e.

There's Always a Bigger Fish.

Purpose: To report a rare case of cancer-associated retinopathy (CAR) presenting initially as a bilateral acute vision loss in a 72-year-old female patient with a history of tongue carcinoma. Systemic work-up revealed a metastatic uterine neuroendocrine carcinoma.

Methods: Case report describing clinical presentation and diagnostic work up in a patient with CAR syndrome.

Deep Immunophenotyping and Clustering Identifies Biomarkers Predictive of Lymphoma in Primary Sjögren Disease.

Objective: Patients with primary Sjögren disease (pSD) are prone to develop non-Hodgkin lymphoma (NHL), but relevant biomarkers are lacking. We aimed to determine new biomarkers predictive of NHL in patients with pSD.

Methods: Two hundred six patients with pSD fulfilling American College of Rheumatology/EULAR 2016 criteria were included and divided into three groups: pSD, lymphoproliferative pSD (Arl-pSD), and NHL-pSD.

Development of a new high-yield integration site assay reveals disease-specific patterns across HTLV-1-associated pathologies.

Human T lymphotropic virus type 1 (HTLV-1) chronic infection is maintained through mitotic proliferation of the infected CD4+ T cells, where the viral genome is integrated as a provirus in its host genome. HTLV-1 integration sites (ISs) have a part in HTLV-1-associated pathologies, with distinct IS patterns associated with malignant proliferation or inflammatory diseases. However, IS determination remains challenging because most assays rely on complex biological and biocomputing protocols.

Diseases associated with anti-centromere protein F (anti-CENP-F) antibodies: Insights from a large cohort study.

Introduction: Antinuclear antibodies (ANA) exhibit diverse specificities and are crucial biomarkers in autoimmune disease assessment. Among ANA, anti-centromere protein-F (CENP-F) antibodies have garnered interest due to their association with malignancies. This study aims to characterize the clinical and biological profiles of a large cohort of anti-CENP-F positive patients and evaluate associated diagnoses.

Impact of conventional and biological disease-modifying anti-rheumatic drugs on arterial lesions in Takayasu arteritis.

Background: The definition of Takayasu arteritis (TAK) remission and disease activity is still unclear. Vascular imaging is an essential tool for following-up patients. Herein, we aimed to compare the evolution of vascular lesions (i.

Screening for Lung Cancer by Chest Computed Tomography During Thromboangiitis Obliterans.

Buerger's disease or thromboangiitis obliterans (TAO) is a non-atherosclerotic inflammatory arteritis strongly associated with smoking exposure. This tobacco use would expose patients to lung cancer. The French-speaking thoracic cancer intergroup recommends screening for lung cancer with a chest computed tomography (CT).

Eosinophilic granulomatosis polyangiitis (EGPA) complicated with periaortitis, precipitating role of dupilumab? A case report a review of the literature.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis that affects small size vessels. Only four cases of periaortitis associated with EGPA have been reported in the literature. We report the case of a 67-year-old woman with EGPA who developed periaortitis 11 months after the initiation of dupilumab for uncontrolled asthma with hypereosinophilia.

CTLA-4 Pathway Is Instrumental in Giant Cell Arteritis.

Background: Giant cell arteritis (GCA) causes severe inflammation of the aorta and its branches and is characterized by intense effector T-cell infiltration. The roles that immune checkpoints play in the pathogenesis of GCA are still unclear. Our aim was to study the immune checkpoint interplay in GCA.

Behçet's Disease Uveitis.

Uveitis in Behçet's disease (BD) is frequent (40% of cases) and is a major cause of morbidity. The age of onset of uveitis is between 20 and 30 years. Ocular involvement includes anterior, posterior, or panuveitis.

Prognosis and long-term outcomes in type I cryoglobulinemia: A multicenter study of 168 patients.

Type I cryoglobulinemia (CG) accounts for 10%-15% of all cryoglobulinemias and are exclusively seen in clonal proliferative hematologic conditions. In this multicenter nationwide cohort study, we analyzed the prognosis and long-term outcomes of 168 patients with type I CG (93 (55.4%) IgM and 75 [44.

Neutrophil, NETs and Behçet's disease: A review.

Behçet's disease (BD) is a chronic systemic vasculitis characterized by recurrent oral and genital ulcers, skin lesions, articular, neurological, vascular and sight-threatening ocular inflammation. BD is thought to share both autoimmune and autoinflammatory disease features. BD is triggered by environmental factors such as infectious agents in genetically predisposed subjects.

Vascular Behçet's disease: a comparative study from Turkey and France.

Objectives: Vascular Behçet's disease (VBD) is a systemic vasculitis involving both arterial and venous vessels of all sizes and occurring in up to 40% of patients with BD. VBD is the main cause of mortality in BD. Although commonly seen around the Mediterranean region, comparative studies in VBD are lacking.

Interferon signature in giant cell arteritis aortitis.

Objectives: Molecular mechanisms underlying large-vessel involvement in giant cell arteritis (LV-GCA) are largely unknown. Herein, we explore the critical involvement of pro-inflammatory signaling pathways in both aorta and T cells from patients with LV-GCA.

Methods: We analyzed transcriptome and interferon gene signature in inflamed aortas from LV-GCA patients and compared them to non-inflammatory control aorta.

Brain Biopsy for Neurological Diseases of Unknown Etiology in Critically Ill Patients: Feasibility, Safety, and Diagnostic Yield.

Objectives: Brain biopsy is a useful surgical procedure in the management of patients with suspected neoplastic lesions. Its role in neurologic diseases of unknown etiology remains controversial, especially in ICU patients. This study was undertaken to determine the feasibility, safety, and the diagnostic yield of brain biopsy in critically ill patients with neurologic diseases of unknown etiology.

Severity, timeline, and management of complications after stereotactic brain biopsy.

Objective: The literature shows discrepancies in stereotactic brain biopsy complication rates, severities, and outcomes. Little is known about the timeline of postbiopsy complications. This study aimed to analyze 1) complications following brain biopsies, using a graded severity scale, and 2) a timeline of complication occurrence.

[Differences of characteristics and outcomes between cancer patients and patients with no active cancer hospitalised for a SARS-CoV-2 infection].

Background: Patients with solid cancer or haematologic malignancies have been considered to be more susceptible to SARS-CoV-2 infection and to more often develop severe complications. We aimed to compare the differences in clinical features and outcomes of COVID-19 patients with and without cancer.

Methods: This was a prospective observational cohort study of consecutive adult patients hospitalised in a COVID-19 unit at Pitié-Salpêtrière Hospital, Paris, France (NCT04320017).

Apremilast in Refractory Behçet's Syndrome: A Multicenter Observational Study.

Objective: Mucocutaneous and joint disorders are the most common manifestations in Behçet's syndrome (BS) and are frequently clustered in the so-called minor forms of BS. There remains a need for safe and effective treatment for joint lesions in BS. We report the long-term safety and effectiveness of apremilast in refractory joint and mucocutaneous manifestations of BS.

Women and Pregnancy in Thromboangiitis Obliterans.

Data regarding women and thromboangiitis obliterans (TAO) are conflicted, and a few cases of pregnancy have been described. We aimed to describe the interplay between TAO and pregnancies. Among 224 TAO patients, 22.