Autoinflammatory diseases in adults. Clinical characteristics and prognostic implications

Rev Clin Esp (Barc). 2017 Mar;217(2):108-116. doi: 10.1016/j.rce.2016.08.004. Epub 2016 Oct 1.
[Article in English, Spanish]

Abstract

Autoinflammatory diseases are clinical conditions with inflammatory manifestations that present in a periodic or persistent manner and are caused by acquired or hereditary disorders of the innate immune response. In general, these diseases are more common in childhood, but cases have been reported in adults and are therefore important for all specialists. There are few references on these diseases in adults due to their low prevalence and underdiagnosis. The aim of this study is to review the scientific literature on these disorders to systematise their clinical, prognostic and treatment response characteristics in adults.

Keywords: Adulto; Adults; Enfermedades autoinflamatorias hereditarias; Fiebre periódica; Hereditary autoinflammatory diseases; Inflamasoma; Inflammasome; Nod-like receptor; Periodic fever; TNF.