Scoping review of biological treatment of deficiency of interleukin-36 receptor antagonist (DITRA) in children and adolescents.

ABSTRACT:

Background

Deficiency of interleukin-36 receptor antagonist (DITRA) is a life threatening monogenic autoinflammatory disease caused by loss of function mutations in the IL36RN gene. Affected patients develop recurrent episodes of generalized pustular psoriasis (GPP) with systemic inflammation and fever. We here review and analyze the literature on pediatric DITRA patients who have been treated by biologicals targeting inflammatory cytokines.

Method

A database research was performed to identify all relevant articles on pediatric DITRA patients treated with biologicals. According to defined response criteria therapeutic efficacy was analyzed.

Results

Our literature research revealed 12 pediatric patients with DITRA who have received treatment with biologicals and we add

SUBMITTER: Hospach T 

PROVIDER: S-EPMC6615208 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature