Objective To develop criteria for the classification of macrophage activation syndrome (MAS)
in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, …

Objective To evaluate the response to treatment of autoinflammatory diseases from an
international registry and an up-to-date literature review. Methods The response to treatment was …

Background To our knowledge, the characteristics and burden of childhood arthritis have
never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study…

Background and aim Familial Mediterranean fever (FMF) is an autoinflammatory disease
caused by mutations of the MEFV gene. We analyse the impact of ethnic, environmental and …

The acronym COACH defines an autosomal recessive condition of Cerebellar vermis hypo/aplasia,
Oligophrenia, congenital Ataxia, Coloboma and Hepatic fibrosis. Patients present the …

Behçet’s disease (BD) and Crohn’s disease (CD) are chronic immune-mediated, inflammatory
disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary …

Background: Interleukin (IL)-1 inhibitors have been suggested as possible therapeutic
options in a large number of old and new clinical entities characterized by an IL-1 driven …

Background Behçet’s disease is a rare multi-systemic inflammatory disease with unknown
etiology which involves principally oral and genital mucosa, skin and eyes. Average age at …

Objectives Autoinflammatory diseases cause systemic inflammation that can result in damage
to multiple organs. A validated instrument is essential to quantify damage in individual …

A few studies have reported the safety profile of interleukin (IL)-1 blockers from real life. The
aim of this study is to describe anakinra (ANA) and canakinumab (CAN) safety profile in …