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

  • Helen J. LachmannEmail author
Chapter
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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).

Keywords

Corticosteroids Monoclonal antibodies Etanercept Infliximab Adalimumab Interleukin-6 Tocilizumab Janus kinase inhibitors Baricitinib Tofacitinib 

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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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.National Amyloidosis CentreUniversity College London and Royal Free Hospital London NHS Foundation TrustLondonUK

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