IgG4-related disease (IgG4-RD) is a rare systemic fibroinflammatory disease frequently
associated with allergy. The pathogenesis of IgG4-RD is poorly understood, and effective …

Deficiency of human adenosine deaminase type 2 (DADA2) is a complex systemic
autoinflammatory disorder characterized by vasculopathy, immune dysregulation, and …

Abstract A20, encoded by TNFAIP3, is a critical negative regulator of immune activation. A20
is a ubiquitin editing enzyme with multiple domains, each of which mediates or stabilizes a …

Background The objective of this initiative was to develop a treat-to-target (T2T) approach for
the management of patients with Familial Mediterranean Fever (FMF), including the …

Autoinflammatory diseases are a group of clinical syndromes characterized by constitutive
overactivation of innate immune pathways. This results in increased production of or …

Autoinflammatory diseases (AIDs) arise from disturbances that alter interactions of immune
cells and tissues. They give rise to prominent (auto) inflammation in the absence of aberrant …

The family of autoinflammatory diseases (AIDs) continues to expand and now includes over
40 genetically defined disorders. Their defining feature is a dysregulated inflammatory …

Primary atopic disorders (PADs) are monogenic diseases characterized by allergy or atopy‐
related symptoms as fundamental features. In patients with PADs, primary immune …

Monogenic lesions in pathways critical for effector functions responsible for immune
surveillance, protection against autoinflammation and appropriate responses to allergens …

Abstract Haploinsufficiency of A20 (HA20) is a rare monogenic disease caused by
heterozygous loss-of-function mutations in the tumor necrosis factor alpha-induced protein 3 …