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Published in: Current Rheumatology Reports 3/2021

01-03-2021 | Vasculitis | Vasculitis (C Dejaco and C Duftner, Section Editors)

Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa

Authors: Alojzija Hočevar, Matija Tomšič, Katja Perdan Pirkmajer

Published in: Current Rheumatology Reports | Issue 3/2021

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Abstract

Purpose of the Review

Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries. Different etiopathogenetic and clinical variants of the disease have been recognized over the past decades. In the present paper, we review the clinical features, diagnosis, and treatment of the different subtypes of the disease.

Recent Findings

The diagnosis of polyarteritis nodosa is primarily based on clinical findings, imaging, and histopathological investigations. Microbiological and genetic investigations complement the diagnostic work-up. Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange.

Summary

PAN is a disease with heterogeneous presentations, severity, and therapeutic approaches. The overall prognosis of this disease is improving, mainly due to early diagnosis and more effective treatments. Treatment choices are guided mainly by the disease subtype and severity. In this review, we have presented the current knowledge on PAN clinical variants, their classification, diagnosis, and treatment approaches.
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Metadata
Title
Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa
Authors
Alojzija Hočevar
Matija Tomšič
Katja Perdan Pirkmajer
Publication date
01-03-2021
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 3/2021
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-021-00983-2

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