RT Journal Article
SR Electronic
T1 Autoinflammatory syndromes in neurology: when our first line of defence misbehaves
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP practneurol-2021-003031
DO 10.1136/practneurol-2021-003031
A1 William K Diprose
A1 Anthony Jordan
A1 Neil E Anderson
YR 2021
UL http://pn.bmj.com/content/early/2021/10/21/practneurol-2021-003031.abstract
AB Autoinflammatory syndromes result from a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis.