B Lineage Cells in ANCA-Associated Vasculitis

Merino-Vico, Ana; Hamburg, Jan Piet van; Tas, Sander W.

doi:10.3390/ijms23010387

Cited by 14 publications

(8 citation statements)

References 154 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Abnormal activation of B cells was observed in AAV patients and was associated with disease severity or poor outcomes. [ 92 , 93 , 94 ] Clinical trials have shown that depletion of B cells with rituximab is a useful treatment for AAV. [ 41 , 42 ] All evidence suggests that B cells have a vital role in the pathogenesis of AAV.…”

Section: Pathogenesismentioning

confidence: 99%

Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis

Sun

Chen

2023

Rheumatology and Immunology Research

View full text Add to dashboard Cite

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses a group of potentially life-threatening disorders characterized by necrotizing small vessel vasculitis with positive serum ANCA. To date, the pathogenesis of AAV has not been fully elucidated, but remarkable progress has been achieved in the past few decades. In this review, we summarize the mechanism of AAV. The pathogenesis of AAV involves various factors. ANCA, neutrophils, and the complement system play key roles in disease initiation and progression, forming a feedback amplification loop leading to vasculitic injury. Neutrophils activated by ANCA undergo respiratory burst and degranulation, as well as releasing neutrophils extracellular traps (NETs), thus causing damage to vascular endothelial cells. Activated neutrophils could further activate the alternative complement pathway, leading to the generation of complement 5a (C5a), which amplifies the inflammatory response by priming neutrophils for ANCA-mediated overactivation. Neutrophils stimulated with C5a and ANCA could also activate the coagulation system, generate thrombin, and subsequently cause platelet activation. These events in turn augment complement alternative pathway activation. Moreover, disturbed B-cell and T-cell immune homeostasis is also involved in disease development. In-depth investigation in pathogenesis of AAV might help to offer more effective targeted therapies.

show abstract

Section: Pathogenesismentioning

confidence: 99%

Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis

Sun

Chen

2023

Rheumatology and Immunology Research

View full text Add to dashboard Cite

show abstract

“…They bind to neutrophils, especially in the small blood vessels. This triggers a cascade of inflammatory reactions, leading to the activation of neutrophils, which release damaging substances such as ROS and lytic enzymes and contribute to the inflammation seen in AAV [44,46,47]. Moreover, a significant contribution to tissue damage is connected with the further formation and release of neutrophil-extracellular traps (NETs) associated with neutrophil apoptosis (NETosis).…”

Section: Pathogenesis and Influence Of Neutrophilic Elements On The D...mentioning

confidence: 99%

The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies

Walulik,

Łysak,

Błaszkiewicz

et al. 2023

IJMS

View full text Add to dashboard Cite

Recent years have brought progress in understanding the role of the neutrophil, dispelling the dogma of homogeneous cells mainly involved in the prime defence against pathogens, shedding light on their pathogenic role in inflammatory diseases and on the importance of antineutrophil-cytoplasmic antibodies’ pathogenic role in ANCA-associated vasculitides vasculitis (AAV). Myeloperoxidase (MPO) and proteinase 3 (PR3) expressed in neutrophil granulocytes are the most common targets for ANCAs and contribute to the formation of MPO-ANCAs and PR3-ANCAs which, released to the bloodstream, become an excellent diagnostic tool for AAV. In this study, we focus on increasing the clinical and experimental evidence that supports the pathogenic role of ANCAs in AAV. Additionally, we discuss the diagnostic utility of ANCAs for disease activity and prognosis in AAV. Understanding the central role of ANCAs in AAV is crucial for advancing our knowledge of these complex disorders and developing targeted therapeutic strategies in the era of personalized medicine.

show abstract

“…ANCAs induce inflammation and endothelial damage, most notably in small‐sized blood vessels in the lungs and kidneys of the patients, eventually leading to organ dysfunction [ 22 ]. The alterations in B cell composition in patients with AAV, the prominent presence of ANCAs, and preliminary data on dysregulated intracellular signalling pathways in these cells suggest that B cells and PCs are critically involved in the pathogenesis of AAV [ 23 ].…”

Section: B Lineage Cells and Their Central Role In Autoimmune Diseasesmentioning

confidence: 99%

Targeting B cells and plasma cells in autoimmune diseases: From established treatments to novel therapeutic approaches

et al. 2022

Self Cite

View full text Add to dashboard Cite

Autoimmune diseases are characterized by the recognition of self-antigens by the immune system, which leads to inflammation and tissue damage. B cells are directly and indirectly involved in the pathophysiology of autoimmunity, both via antigen-presentation to T cells and production of proinflammatory cytokines and/or autoantibodies. Consequently, B lineage cells have been identified as therapeutic targets in autoimmune diseases. B cell depleting strategies have proven beneficial in the treatment of rheumatoid arthritis (RA), systemic lupus erythematous (SLE), ANCA-associated vasculitis (AAV), multiple sclerosis (MS), and a wide range of other immune-mediated inflammatory diseases (IMIDs). However, not all patients respond to treatment or may not reach (drug-free) remission. Moreover, B cell depleting therapies do not always target all B cell subsets, such as short-lived and long-lived plasma cells. These cells play an active role in autoimmunity and in certain diseases their depletion would be beneficial to achieve disease remission. In the current review article, we provide an overview of novel strategies to target B lineage cells in autoimmune diseases, with the focus on rheumatic diseases. Both advanced therapies that have recently become available and more experimental treatments that may reach the clinic in the near future are discussed.

show abstract

B Lineage Cells in ANCA-Associated Vasculitis

Cited by 14 publications

References 154 publications

Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis

Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis

The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies

Targeting B cells and plasma cells in autoimmune diseases: From established treatments to novel therapeutic approaches

Contact Info

Product

Resources

About