Zhengping Huang1, Tianwang Li2, Peter A Nigrovic3, Pui Y Lee4. 1. Department of Rheumatology and Immunology, Guangdong Second Provincial General Hospital, Guangzhou, China; Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA. 2. Department of Rheumatology and Immunology, Guangdong Second Provincial General Hospital, Guangzhou, China. 3. Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA; Division of Immunology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. 4. Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA; Division of Immunology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. Electronic address: pui.lee@childrens.harvard.edu.
Abstract
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized arteries. With the establishment and refinement of vasculitis nomenclature and diagnostic criteria, clinical findings of PAN and distinguishing features from other vasculitides are now well characterized. Although PAN typically manifests in adulthood, cohort studies in paediatric patients have shaped our understanding of childhood-onset PAN. The paradigm of childhood-onset PAN changed considerably with the landmark discovery of deficiency of ADA2 (DADA2), a monogenic cause of vasculitis that is often indistinguishable from PAN. Testing for DADA2 has provided an explanation to numerous challenging cases of familial PAN and early-onset PAN around the world. The ability to distinguish DADA2 from classic PAN have important therapeutic implications as tumor necrosis factor inhibitors have demonstrated remarkable efficacy in the treatment of DADA2. In this review, we will discuss our current understanding of PAN and DADA2 and highlight similarities and differences between these vasculitides.
Polyarteritis nodosa (PAN) is a systemic n class="Disease">necrotizing vasculitis that predominantly affects medium-sized arteries. With the establishment and refinement of vasculitis nomenclature and diagnostic criteria, clinical findings of PAN and distinguishing features from other vasculitides are now well characterized. Although PAN typically manifests in adulthood, cohort studies in paediatric patients have shaped our understanding of childhood-onset PAN. The paradigm of childhood-onset PAN changed considerably with the landmark discovery of deficiency of ADA2 (DADA2), a monogenic cause of vasculitis that is often indistinguishable from PAN. Testing for DADA2 has provided an explanation to numerous challenging cases of familial PAN and early-onset PAN around the world. The ability to distinguish DADA2 from classic PAN have important therapeutic implications as tumor necrosis factor inhibitors have demonstrated remarkable efficacy in the treatment of DADA2. In this review, we will discuss our current understanding of PAN and DADA2 and highlight similarities and differences between these vasculitides.
Authors: Andrey V Zavialov; Eduard Gracia; Nicolas Glaichenhaus; Rafael Franco; Anton V Zavialov; Grégoire Lauvau Journal: J Leukoc Biol Date: 2010-05-07 Impact factor: 4.962
Authors: J C Jennette; R J Falk; K Andrassy; P A Bacon; J Churg; W L Gross; E C Hagen; G S Hoffman; G G Hunder; C G Kallenberg Journal: Arthritis Rheum Date: 1994-02
Authors: Kristen M Gibson; Kimberly A Morishita; Paul Dancey; Paul Moorehead; Britt Drögemöller; Xiaohua Han; Jinko Graham; Robert E W Hancock; Dirk Foell; Susanne Benseler; Rashid Luqmani; Rae S M Yeung; Susan Shenoi; Marek Bohm; Alan M Rosenberg; Colin J Ross; David A Cabral; Kelly L Brown Journal: Arthritis Rheumatol Date: 2019-08-26 Impact factor: 10.995
Authors: Hasan Hashem; Ashish R Kumar; Ingo Müller; Florian Babor; Robbert Bredius; Jignesh Dalal; Amy P Hsu; Steven M Holland; Dennis D Hickstein; Stephen Jolles; Robert Krance; Ghadir Sasa; Mervi Taskinen; Minna Koskenvuo; Janna Saarela; Joris van Montfrans; Keith Wilson; Barbara Bosch; Leen Moens; Michael Hershfield; Isabelle Meyts Journal: Blood Date: 2017-10-03 Impact factor: 22.113
Authors: Pui Y Lee; Yuelong Huang; Qing Zhou; Oskar Schnappauf; Michael S Hershfield; Ying Li; Nancy J Ganson; Natalia Sampaio Moura; Ottavia M Delmonte; Scellig S Stone; Michael J Rivkin; Sung-Yun Pai; Todd Lyons; Robert P Sundel; Victor W Hsu; Luigi D Notarangelo; Ivona Aksentijevich; Peter A Nigrovic Journal: J Allergy Clin Immunol Date: 2018-06-21 Impact factor: 10.793