Autoinflammatory diseases are conditions in which pathogenic inflammation arises primarily
through antigen-independent hyperactivation of immune pathways. First recognized just …

Monogenic autoinflammatory diseases are a group of rheumatologic disorders caused by
dysregulation in the innate immune system. The molecular mechanisms of these disorders …

Background Deficiency of adenosine deaminase 2 (DADA2) is a syndrome with pleiotropic
manifestations including vasculitis and hematologic compromise. A systematic definition of …

The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited
disease that has undergone extensive phenotypic expansion since being first described in …

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic disease caused by biallelic
mutations in ADA2 gene (previously CECR1). The aim of this review was to describe the …

Importance Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited
disease characterized by systemic vasculitis, early-onset stroke, bone marrow failure, and/or …

Objective Deficiency of adenosine deaminase 2 (DADA2) is a potentially fatal monogenic
syndrome characterized by variable manifestations of systemic vasculitis, bone marrow …

Deficiency of adenosine deaminase 2 (DADA2) was first described as a monogenic form of
systemic vasculitis that closely resembles polyarteritis nodosa (PAN). The phenotypic …

Systemic autoinflammatory diseases (SAIDs) are a heterogeneous group of disorders
caused by excess activation of the innate immune system in an antigen-independent …

Objective To evaluate the impact of anti-Tumour Necrosis Factor-α (anti-TNF) treatment on
the occurrence of vasculitic ischaemic events in patients with deficiency of adenosine …