Translational implications of humoral and cellular immune dysfunction in granulomatosis with polyangiitis.

Cytokine

Immunogenomics Laboratory, Department of Human Genetics and Molecular Medicine, School of Health Sciences, Central University of Punjab, Bathinda, India. Electronic address:

Published: April 2023

AI Article Synopsis

  • Granulomatosis with polyangiitis (GPA) is a rare and serious autoimmune vasculitis linked to ANCA antibodies, with increasing prevalence, especially in developing countries.
  • The disease is triggered in genetically predisposed individuals by external stimuli, leading to an abnormal immune response characterized by harmful interactions between immune cells and endothelial cells.
  • Current research focuses on understanding the complex immune mechanisms behind GPA to develop effective diagnostic tools and treatments, including new monoclonal antibodies for safer management and longer remission periods.

Article Abstract

Granulomatosis with polyangiitis (GPA) is a rare systemic ANCA (Anti-neutrophil cytoplasmic antibodies) associated vasculitis (AAV). In the last couple of decades, GPA has emerged as a disease of concern due to rapid increase in the prevalence and incidence especially in developing countries. Unknown aetiology and rapid progression have made GPA a critical disease. Thus, establishing specific tools to facilitate early and faster disease diagnosis and efficient disease management has immense importance. GPA may develop in genetically predisposed individuals on receiving the external stimulus (i.e. microbial pathogen, pollutant etc.) that triggers the immune response. B-cell activating factor (BAFF) produced by the neutrophils, promotes the B-cell maturation and survival which leads to increased ANCA production. Abnormal B-cell and T-cell proliferation and their cytokine response plays a major role in disease pathogenesis and granuloma formation. ANCA interacts with neutrophils and induces the neutrophil extracellular traps (NETs) formation and reactive oxygen species (ROS) production which leads to the endothelial cell injury. This review article summarizes the critical pathological events and how cytokines and immune cells shape the GPA pathogenesis. Decoding this complex network would facilitate in developing tools for diagnosis, prognosis and disease management. Recently developed specific monoclonal antibodies (MAbs) targeting cytokines and immune cells are being used for safer treatment and achieving longer remission.

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Source
http://dx.doi.org/10.1016/j.cyto.2023.156154DOI Listing

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