Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic
diseases. The condition is considered part of secondary hemophagocytic …

Objective Systemic juvenile idiopathic arthritis (JIA) is associated with high levels of
interleukin‐6 (IL‐6) in the serum and synovial fluid, and impairment of natural killer (NK) cell …

Objectives Interferon-γ (IFNγ) is the pivotal mediator in murine models of primary
haemophagocytic lymphohistiocytosis (pHLH). Given the similarities between primary and …

Disclosures: P. Izmirly reported personal fees from GlaxoSmithKline outside the submitted
work. JP Buyon reported personal fees from Bristol-Myers Squibb LN advisory board …

Nerve growth factor (NGF) levels are highly increased in inflamed tissues, but their role is
unclear. We show that NGF is part of a regulatory loop in monocytes: inflammatory stimuli …

Background The pathogenesis of macrophage activation syndrome (MAS) is not clearly
understood: a large body of evidence supports the involvement of mechanisms similar to …

CD8+ T-cell activation has been demonstrated to distinguish patients with primary and
infection-associated hemophagocytic lymphohistiocytosis (HLH) from patients with early …

Intralysosomal cystine crystal accumulation, due to mutations in the CTNS gene, is a
hallmark of nephropathic cystinosis, but the role of these crystals in disease pathogenesis …

The role of Interleukin (IL)-6 in the pathogenesis of joint and systemic inflammation in
rheumatoid arthritis (RA) and systemic juvenile idiopathic arthritis (s-JIA) has been clearly …

Objective. An upregulation of type I interferon (IFN) stimulated genes [IFN score (IS)] was
described in patients with adenosine deaminase 2 deficiency (DADA2). We describe the …