Karen L Vega Villanueva1, Luis R Espinoza2. 1. Section of Rheumatology, LSU Health Sciences Center, New Orleans, LA, 70112, USA. 2. Section of Rheumatology, LSU Health Sciences Center, New Orleans, LA, 70112, USA. lespin1@lsuhsc.edu.
Abstract
PURPOSE OF REVIEW: Eosinophilic granulomatosis with polyangiitis (EGPA) represents a rare clinical entity, which is getting increasing attention and relevance in view of our better understanding and newer insights into its pathogenesis. Concomitantly better recognition and understanding of the immune pathophysiologic role of eosinophils provide a solid ground of their role on systemic inflammatory disorders and defense against infectious triggers, especially parasites. This review will focus on describing the physiopathology of eosinophils, as well as providing an in depth description of the natural history, clinical spectrum, and therapy of EGPA. RECENT FINDINGS: Several studies have aimed at finding useful biomarkers to monitor disease activity, and reported data have shown that eotaxin 3, IL25, IL33, and some eicosanoids to be promising options. Regarding therapeutic advances, recently published studies have revealed the efficacy of mepolizumab during induction and maintenance of EGPA. Recently published data confirmed earlier studies that the use of azathioprine during the induction phase is of no benefit during long-term follow-up. In addition, data from the REOVAS study, which uses rituximab, is still ongoing and apparently with promising results. Eosinophils are involved in several systemic inflammatory disorders, and recent gathered data provide support for their role in triggering EGPA. Better understanding of its pathophysiology should generate newer insights into the pathogenesis, biomarkers of disease activity, and therapeutic targets.
PURPOSE OF REVIEW: Eosinophilic granulomatosis with polyangiitis (EGPA) represents a rare clinical entity, which is getting increasing attention and relevance in view of our better understanding and newer insights into its pathogenesis. Concomitantly better recognition and understanding of the immune pathophysiologic role of eosinophils provide a solid ground of their role on systemic inflammatory disorders and defense against infectious triggers, especially parasites. This review will focus on describing the physiopathology of eosinophils, as well as providing an in depth description of the natural history, clinical spectrum, and therapy of EGPA. RECENT FINDINGS: Several studies have aimed at finding useful biomarkers to monitor disease activity, and reported data have shown that eotaxin 3, IL25, IL33, and some eicosanoids to be promising options. Regarding therapeutic advances, recently published studies have revealed the efficacy of mepolizumab during induction and maintenance of EGPA. Recently published data confirmed earlier studies that the use of azathioprine during the induction phase is of no benefit during long-term follow-up. In addition, data from the REOVAS study, which uses rituximab, is still ongoing and apparently with promising results. Eosinophils are involved in several systemic inflammatory disorders, and recent gathered data provide support for their role in triggering EGPA. Better understanding of its pathophysiology should generate newer insights into the pathogenesis, biomarkers of disease activity, and therapeutic targets.
Authors: Jonathan Steinfeld; Eric S Bradford; Judith Brown; Stephen Mallett; Steven W Yancey; Praveen Akuthota; Maria C Cid; Gerald J Gleich; David Jayne; Paneez Khoury; Carol A Langford; Peter A Merkel; Frank Moosig; Ulrich Specks; Peter F Weller; Michael E Wechsler Journal: J Allergy Clin Immunol Date: 2018-12-19 Impact factor: 10.793
Authors: Xavier Puéchal; Christian Pagnoux; Gabriel Baron; François Lifermann; Loïk Geffray; Thomas Quémeneur; Jean-Luc Saraux; Marie Wislez; Vincent Cottin; Marc Ruivard; Nicolas Limal; Achille Aouba; Bernard Bonnotte; Antoine Néel; Christian Agard; Pascal Cohen; Benjamin Terrier; Claire Le Jeunne; Luc Mouthon; Philippe Ravaud; Loïc Guillevin Journal: Rheumatology (Oxford) Date: 2019-12-01 Impact factor: 7.580
Authors: Jesse C Nussbaum; Steven J Van Dyken; Jakob von Moltke; Laurence E Cheng; Alexander Mohapatra; Ari B Molofsky; Emily E Thornton; Matthew F Krummel; Ajay Chawla; Hong-Erh Liang; Richard M Locksley Journal: Nature Date: 2013-09-15 Impact factor: 49.962