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

Autoimmunity as a continuum in primary immunodeficiency : Current Opinion in Pediatrics
Autoimmunity as a continuum in primary immunodeficiency : Current Opinion in Pediatrics Log …

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

Primary immune regulatory disorders (PIRD) are associated with autoimmunity,
autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune …

Purpose Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of
immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo …

Abstract Purpose of Review We aim to describe the pathophysiology, clinical findings,
diagnosis, and treatment of deficiency of adenosine deaminase 2 (DADA2). Recent Findings …

Background Deficiency of adenosine deaminase 2 (DADA2) is an autoinflammatory disease
caused by deleterious ADA2 variants. The frequency of these variants in the general …

Purpose Deficiency of adenosine deaminase type 2 (ADA2)(DADA2) is a rare inborn error of
immunity caused by deleterious biallelic mutations in ADA2. Clinical manifestations are …

Background: Primary Immunodeficiencies (PIDs) are a heterogeneous group of genetic
immune disorders. While some PIDs can manifest with more than one phenotype, signs, and …

Introduction Deficiency of adenosine deaminase 2 (DADA2) is a rare monogenic
autoinflammatory disease, whose clinical phenotype was expanded since the first cases …