@inbook{baa250433edd4d6ab6fc238b1b38dfb7,
title = "Systemic Polyarteritis Nodosa",
abstract = "Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis predominantly affecting the medium-sized arteries with widely variable presenting features, disease course, and outcomes. Recent updates regarding the nomenclature of PAN have resulted in the description of several PAN sub-phenotypes. Herein discussed are idiopathic PAN, Hepatitis B-associated PAN and monogenic disorders such as adenosine deaminase-2 deficiency. The current understanding of the pathogenesis, histopathological features, and treatment of these conditions are reviewed.",
keywords = "Adenosine deaminase 2 deficiency, Autoimmunity, Hepatitis B, Polyarteritis nodosa, Vasculitis",
author = "Koster, {Matthew J.}",
note = "Publisher Copyright: {\textcopyright} 2021, Springer Nature Switzerland AG.",
year = "2021",
doi = "10.1007/978-3-030-67175-4_14",
language = "English (US)",
series = "Rare Diseases of the Immune System",
publisher = "Springer Nature",
pages = "161--179",
booktitle = "Rare Diseases of the Immune System",
address = "United States",
}