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Giornale Italiano di Dermatologia e Venereologia 2020 Jul 02

DOI: 10.23736/S0392-0488.20.06708-5

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

How to handle the main drugs to treat autoinflammatory disorders and how we treat common autoinflammatory diseases

Isabelle KONÉ-PAUT 1, 2 , Perrine DUSSER 1, 2

1 Division of Pediatric Rheumatology, Bicêtre Hospital, APHP, University of Paris Sud Saclay, Le Kremlin-Bicêtre, France; 2 CEREMAIA, Bicêtre Hospital, APHP, University of Paris Sud Saclay, Le Kremlin-Bicêtre, France


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This article provides an overview of the main drugs to treat autoinflammatory disorders focusing on the four emblematic diseases within this group which represent, to date, the vast majority of patients with monogenic SAID; i.e. familial Mediterranean fever, mevalonate kinase deficiency, TNF receptor 1 deficiency and cryopyrin-associated periodic syndrome. We will therefore resume the evolutionary risks of the four main IL-1 dependent SAID, there treatments and monitoring tools. After having exposed the general principles, we will detail specific guidelines for the management in everyday clinical practice of patients according to the four main pathologies based on both our expertise and international recommendations. We aim herein to guide practitioners in charge of patients with common SAID towards optimal follow-up with appropriate monitoring of anti-inflammatory drugs.


KEY WORDS: Familial Mediterranean fever; Mevalonate kinase deficiency; Type I tumor necrosis factor receptors; Cryopyrin-associated periodic syndrome; Hereditary autoinflammatory diseases

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