期刊
CLINICAL IMMUNOLOGY
卷 215, 期 -, 页码 -出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2020.108411
关键词
Polyarteritis nodosa; Adenosine deaminase 2; DADA2; Vasculitis
类别
资金
- Rheumatology Research Foundation Investigator Award
- Boston Children's Hospital Faculty Career Development Award
- National Institute of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) [K08-AR074562, R01-AR065538, R01-AR073201, R01-AR075906, P30-AR070253]
- Fundacion Bechara
- Arbuckle Family Fund for Arthritis Research
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.
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