Результаты поиска

Current State of Knowledge on Primary Sjögren's Syndrome, an Autoimmune Exocrinopathy.

Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most frequent connective tissue disease after rheumatoid arthritis, affecting 0.3-3% of the population. Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the saliv…

Treating rheumatic patients with a malignancy.

Management of patients with inflammatory rheumatic disease and a history of (or even a current) malignant disease poses some particular challenges. As direct evidence of the risk of (recurrent or de novo) malignancy in patients with a history of malignant disease is scarce, such a risk may be estimated indirectly from the principal carcinogenicity of the respective drug to be used or (also indirectly) from cancer reactivation data from the transplant literature. In general, cancer risk is increased in patients receiving combination immunosuppressive treatment, but the risk in patients receivin…

Sjögren Disease-B Cells at the Brink: From Autoimmunity to Lymphomagenesis and the Rise of Novel B Cell-Targeted Therapies.

Sjögren disease (SjD) is a common systemic autoimmune disorder characterized by inflammation of the exocrine glands, resulting in dryness. P … years of recurrent glandular swelling-highlighting a common yet challenging scenario for practicing rheumatologists. Using this case as a framework, we examine the pathobiology of B cells in SjD that drive autoreactivity and lymphomagenesis. Finally, we review emerging B cell-targeted therapies that reflect a broader shift in the SjD treatment landscape …

Relapsing polychondritis: tracheobronchial involvement and differential diagnoses.

This review is describing the diagnostic and therapeutic approach to tracheobronchial involvement in relapsing polychondritis (RP), with a focus on differential diagnoses of inflammatory origin. RP is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the involved cartilages. Besides the damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are the most severe, and can seriously alter the prognosis. Tracheobronchial lesions are assesse

Совпадение в полном тексте источника
… Semin Arthritis Rheum 2021;51:339-46. 10.1016/j.semarthrit.2021.02.002 53. Terrier B, Dechartres A, Girard C, et al. Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience. … Semin Arthritis Rheum 2012;41:712-9. 10.1016/j.semarthrit.2011.08.006 88. Moulis G, Pugnet G, Costedoat-Chalumeau N, et al. Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study. Ann Rheum Dis 2018;77:1172-8. 10.1136/annrheumdis-2017-212705 89. …

Extrahepatic Manifestations of Chronic HBV Infection and the Role of Antiviral Therapy.

The hepatitis B virus (HBV) infection leads to chronic hepatitis, cirrhosis, and hepatocarcinoma. However, about 20% of patients experience extrahepatic manifestations such as polyarteritis nodosa, non-rheumatoid arthritis, non-Hodgkin lymphoma, cryoglobulinemic vasculitis, and glomerulonephritis. These influence the patient's morbidity, quality of life and mortality. The treatment of an HBV infection is based on nucleotide analogues (NAs) which are safe and effective for the suppression of HBV-DNA in almost 100% of cases. A few studies have shown that NAs induce a viral response and an improv…

Case report: Single infusion of combined anti-CD20 and anti-CD38 monoclonal antibodies in pediatric refractory lupus nephritis.

Lupus nephritis (LN), present in 30%-50% of systemic lupus erythematosus (SLE) patients, often necessitates standard immunosuppressive therapy (glucocorticoids, MMF, CYC) as suggested by the European League Against Rheumatism/European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) and Kidney Disease Improving Global Outcomes (KDIGO) guidelines. However, a subset of subjects remains refractory. Recent findings suggested the efficacy of targeting CD38-long-lived plasma cells in LN and SLE refractory to standard treatment. However, previous experiences were limite…

Системный склероз-ассоциированная легочная гипертензия: спектр и воздействие

Систематическая склерозная гипертония легких (SSc-PAH) является катастрофическим осложнением one из наиболее распространенных и разрушительных аутоиммунных заболеваний. После диагностирования он становится главной причиной смертности среди этой пациентки. Различные организации и общества разработали и подтвердили методы обследования и оценки рисков, с тем чтобы выявить пациентов на раннем этапе их курса лечения и незамедлительно направить их в экспертные центры для гемодинамической оценки и официальной диагностики. Кроме того, в настоящее время в ряде крупных многоцентровых клинических испытан

Совпадение в полном тексте источника
… Arthritis Rheum. 2003;48:516–522. doi: 10.1002/art.10775. 27. Steen V.D. Autoantibodies in Systemic Sclerosis. Semin. Arthritis Rheum. 2005;35:35–42. doi: 10.1016/j.semarthrit.2005.03.005. 28. … Rheum. Dis. 2013;73:1340–1349. doi: 10.1136/annrheumdis-2013-203301. 51. …

Daratumumab in systemic lupus erythematosus: a single-arm phase 2 trial.

Antibody-secreting cells (ASCs) play a central role in the pathophysiology of systemic lupus erythematosus (SLE). This single-arm, open-label, phase 2 clinical trial aims to evaluate the safety and efficacy of the ASC-depleting anti-CD38 monoclonal antibody daratumumab in patients with SLE (NCT04810754). The primary endpoint is the reduction in serum anti-double-stranded DNA (anti-dsDNA) antibody levels at week 12. Key secondary end points include safety, clinical efficacy, and immunologic changes. Ten female patients with active disease and inadequate responses to at least two immunosuppressi

Совпадение в полном тексте источника
… Ann. rheum. dis. 75 , 1407–1415 (2016). 38. Ohl, K. & Tenbrock, K. Regulatory T cells in systemic lupus erythematosus. Eur. J. immunol. 45 , 344–355 (2015). 39. Sumida, T. S., Cheru, N. T. & Hafler, D. A. … Rheum. Dis. 82 , 1497–1499 (2023). 48. Janssen Biotech. Dosing and Administration Guide Darzalex Faspro https://www.darzalexhcp.com/pdfs/dosing-and-administration-guide-faspro.pdf accessed 18.11.2025. 49. …

Renal Involvement in Mixed Cryoglobulinemic Vasculitis: Current Perspectives.

Cryoglobulinemia is a rare disorder characterized by the presence of abnormal proteins (cryoglobulins) in the blood that precipitate at low temperatures. It presents with a wide clinical spectrum, from mild symptoms to severe, life-threatening disease. In mixed cryoglobulinemia (MC), vascular damage results from immune complexes-typically monoclonal IgM with rheumatoid factor activity and polyclonal IgG (Type II), or polyclonal/oligoclonal IgM and IgG (Type III). These complexes can obstruct small blood vessels, leading to ischemia and leukocytoclastic vasculitis. Renal involvement occurs in a…

Polymyalgia rheumatica: An update (Review).

Polymyalgia rheumatica (PMR) is a chronic inflammatory disease which affects the connective vascular tissue, characterized by pain accompanied by morning stiffness, predominantly of the neck muscles, hip and shoulder girdle. Usually, patients with this disease are >50 years of age and biological inflammatory syndrome is present with an increase in both the erythrocyte sedimentation rate and C-reactive protein levels, aspects similar to giant cell arteritis. The aim of the present review was to depict the current pathogenic hypothesis, diagnostic and treatment approach for patients with PMR, an…

Chimeric Antigen Receptor T-Cell Revolution Remodeling Immunity to Conquer Autoimmune Disease.

Chimeric antigen receptor T (CAR-T) cell therapy, a groundbreaking technology in tumor immunotherapy, has demonstrated unprecedented potential in the field of autoimmune diseases in recent years. This article provides a systematic review of the developmental trajectory and core concepts of CAR-T-cell therapy in autoimmune diseases, emphasizing its conceptual evolution from traditional "killing" strategies to "precision immune remodeling". Leveraging multitarget approaches (e.g., CD19 and B-cell maturation antigen) and chimeric autoantigen receptor technology, it achieves efficient elimination

Совпадение в полном тексте источника
… Semin Arthritis Rheum. 2024;67: Article 152479. 8. Tur C, Eckstein M, Velden J, Rauber S, Bergmann C, Auth J, Bucci L, Corte G, Hagen M, Wirsching A, et al. CD19-CAR T-cell therapy induces deep tissue depletion of B cells. Ann Rheum Dis. 2025;84(1):106–114. 9. … Ann Rheum Dis. 2024;83(Suppl 1):1131–1132. 69. Mamo T, Dreyzin A, Stroncek D, McKenna DH. Emerging biomarkers for monitoring chimeric antigen receptor T-cell therapy. Clin Chem. 2024;70(1):116–127. 70. …

Seventeen-year reassessment of diagnostic transitions, biologic therapy initiation and mortality in spondyloarthritis: results from the REGISPON-3 study.

OBJECTIVES: To evaluate disease evolution, diagnostic transitions, time to biologic treatment initiation and mortality in patients with spon … l and treatment data were collected and compared with baseline. Diagnostic changes according to the rheumatologist's judgement and their associated factors were analysed using multivariable logistic regression. Time to initiation of biologic therapy was evaluated using Kaplan-Meier survival analysis. RESULTS: A total of 536 patients from the REGISPONSE …

Role of T cells and cytokines in the pathogenesis of rheumatoid arthritis.

Rheumatoid arthritis (RA) is a long-term autoimmune disease. It causes persistent joint inflammation, aberrant tissue growth, and progressive joint deterioration. T cells have a key role in the onset of RA. They identify and trigger other immune cells, and produce inflammatory signals such as interleukin-6 (IL-6), interleukin-17 (IL-17), and tumor necrosis factor-α (TNF-α). Prolonged inflammation and tissue damage are caused by an imbalance between regulatory T cells (Tregs) and pro-inflammatory T helper 17 (Th17) cells. Additionally, the condition is exacerbated by uncontrolled cytokine netwo…

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.

OBJECTIVE: Vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality. METHODS: Given the variability in disease presentation and the limited number of studies to date, no clinical documents currently exist to provide guidance to health care providers about the management of VEXAS. To address this gap, we formed an international multidisciplinary panel of VEXA

Совпадение в полном тексте источника
… Semin Arthritis Rheum 2024 ; 66 : 152430 . 38554594 10.1016/j.semarthrit.2024.152430 7 Tan IJ , Ferrada MA , Ahmad S , et al. Skin Manifestations of VEXAS syndrome and associated genotypes . … Ann Rheum Dis 2024 ; 83 ( 3 ): 372 – 381 . 38071510 10.1136/ard-2023-224819 44 Kusne Y , Ghorbanzadeh A , Dulau‐Florea A , et al. Venous and arterial thrombosis in patients with VEXAS syndrome . …

Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation.

BACKGROUND: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. Treatment with autologous hematopoietic cell transplantation (HCT) for progressive SSc has improved overall and event-free survival rates significantly, but unfortunately disease progression after HCT is seen in a subset of patients. Data on the efficacy and safety of second HCT is scarce. CASE: We present a patient with diffuse cutaneous SSc and associated interstitial lung disease (ILD) who successfully underwent a second HCT for prog

Совпадение в полном тексте источника
… Ann Rheum Dis (2017) 76:1897–905. doi: 10.1136/annrheumdis-2017-211448 2. Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, et al. … Ann Rheum Dis (2009) 68(1):94–8. doi: 10.1136/ard.2007.082495 25. Smith V, Herrick AL, Ingegnoli F, Damjanov N, De Angelis R, Denton CP, et al. Standardisation of nailfold capillaroscopy for the assessment of patients with raynaud's phenomenon and systemic sclerosis. …

Rheumatoid Arthritis Real-world Cohort Study in China (ReALSA): protocol for a multicentre prospective, longitudinal cohort study.

INTRODUCTION: The burden of rheumatoid arthritis (RA) is profound, although treated with the treat-to-target strategy for RA patients according to the two most influential organisations for rheumatology worldwide. The need to timely achieve the control of disease activity for RA patients, especially for those difficult-to-treat individuals, is still unmet. Besides, the data on the diagnosis and prognosis of RA-related complications or comorbidities such as sarcopenia, cardiovascular diseases (CVD), malignancies and infections in large real-world cohorts are still limited. Therefore, the aim of…

Systemic Sclerosis and Malignancy: A Review of Current Data.

Systemic sclerosis (SSc) is associated with increased risk of malignancy. The organ systems most commonly affected are the lungs, the breast … the importance of the need for more studies on the association of SSc and cancer and should incite rheumatology colleges to develop specific recommendations for the clinician to follow while approaching patients with SSc.

16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends.

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive ther

Совпадение в полном тексте источника
… Ann Rheum Dis 2019 ; 78 : 1296 – 1304 . 31092409 10.1136/annrheumdis-2019-215213 PMC11034817 17 Cohen H Hunt BJ Efthymiou M , et al . … Ann Rheum Dis 2011 ; 70 : 145 – 150 . 20980705 10.1136/ard.2010.134817 89 Andreoli L Piantoni S Dall’Ara F Allegri F Meroni P Tincani A. Vitamin D and antiphospholipid syndrome . …

One Giant Step for Giant Cell Arteritis: Updates in Diagnosis and Treatment.

Purpose of reviewGiant cell arteritis (GCA), a medium and large vessel vasculitis occurring in the aged, remains a formidable disease, capable of taking both vision and life, through a multitude of vascular complications. Our understanding of the spectrum of its manifestations has grown over the years, to include limb claudication, aortitis, and cardiac disease, in addition to the more classic visual complications resulting from of ischemia to branches of the external and internal carotid arteries. While a clinical presentation of headache, jaw claudication, scalp tenderness, fever and other s

Совпадение в полном тексте источника
… Arthritis Rheum. 1998;41:1497–1504. doi: 10.1002/1529-0131(199808)41:8<1497::AID-ART22>3.0.CO;2-Z. 74. … Ann Rheum Dis. 2020;79(1):19–30. doi:10.1136/annrheumdis-2019-215672. …

Molecular therapies for systemic lupus erythematosus: clinical trials and future prospects.

The prognosis of patients with systemic lupus erythematosus has greatly improved since treatment regimens combining corticosteroids and immunosuppressive medications have been widely adopted in therapeutic strategies given to these patients. Immune suppression is evidently efficient but also leads to higher susceptibility to infectious and malignant diseases. Toxic effects and sometimes unexpectedly dramatic complications of current therapies have been progressively reported. Identifying novel molecular targets therefore remains an important issue in the treatment of lupus. The aim of this rev

Совпадение в полном тексте источника
… Arthritis Rheum. 2008;58:2470–2480. doi: 10.1002/art.23673. Jones DS, Barstad PA, Feild MJ, Hachmann JP, Hayag MS, Hill KW, Iverson GM, Livingston DA, Palanski MS, Tibbets AR, Yu L, Coutts SM. … Arthritis Rheum. 2001;44:432–441. doi: 10.1002/1529-0131(200102)44:2<432::AID-ANR62>3.0.CO;2-S. Zinger H, Eilat E, Meshorer A, Mozes E. …