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Corticosteroid, Other Biologic and Small Molecule Therapies in Systemic Autoinflammatory Disorders

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Textbook of Autoinflammation

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Abstract

Not all patients with autoinflammatory disorders respond to treatment with colchicine or anti-interleukin (IL)-1 agents. Although no other biologics nor small molecule pharmaceuticals are currently licenced for use in autoinflammatory disease several agents are used. The pharmacology, mechanisms of action and safety data of these medications are summarised in this chapter. These include corticosteroids, tumor necrosis factor (TNF), IL-6 and Janus kinase (JAK) inhibitors. There are published consensus criteria which provide guidance to the management of familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor associated periodic syndrome (TRAPS) and mevalonate kinase deficiency (MKD).

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Abbreviations

CANDLE:

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature

CAPS:

Cryopyrin-associated periodic syndromes

CAR:

Chimeric antigen receptors

CNO:

Chronic non-bacterial osteomyelitis

CRP:

C-reactive protein

DADA2:

Deficiency of adenosine deaminase 2

FDA:

Food and Drug Administration

FMF:

Familial Mediterranean fever

GM-CSF:

Granulocyte-macrophage colony-stimulating factor

GTI:

Glucocorticoid Toxicity Index

IL:

Interleukin

INF:

Interferon

JAK:

Janus kinase

MKD:

Mevalonate kinase deficiency

NF-κB:

Nuclear factor kappa B

NLRP:

Nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain

NSAIDs:

Nonsteroidal anti-inflammatory drugs

PAPA:

Pyogenic arthritis, pyoderma gangrenosum, acne

PFAPA:

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis

SAA:

Serum amyloid A

SAVI:

STING-associated vasculopathy with onset in infancy

SHARE:

Single Hub and Access point for Paediatric Rheumatology in Europe

SOSC:

Suppressor of cytokine signaling

STAT:

Signal transducer and activator of transcription

TNF:

Tumor necrosis factor

TRAPS:

Tumor necrosis factor receptor-associated periodic syndrome

TYK:

Tyrosine kinase

UK:

United Kingdom

USA:

United States of America

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Authors and Affiliations

  1. National Amyloidosis Centre, University College London and Royal Free Hospital London NHS Foundation Trust, London, UK

    Helen J. Lachmann

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  1. Helen J. Lachmann
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Correspondence to Helen J. Lachmann .

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Editors and Affiliations

  1. Pediatric Rheumatology Unit, Shaare Zedek Medical Center and Hebrew University, Jerusalem, Israel

    Philip J. Hashkes

  2. Department Paediatrics, Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Ronald M. Laxer

  3. Department of Internal Medicine, Radboudumc Expertisecenter for Immunodeficiency and Autoinflammation, Radboud University Medical Center, Nijmegen, The Netherlands

    Anna Simon

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Lachmann, H.J. (2019). Corticosteroid, Other Biologic and Small Molecule Therapies in Systemic Autoinflammatory Disorders. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_42

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  • DOI: https://doi.org/10.1007/978-3-319-98605-0_42

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