Effective treatment of TNFα inhibitors in Chinese patients with Blau syndrome.

ABSTRACT:

Objectives

Blau syndrome (BS) is a rare dominantly inherited autoinflammatory disorder associated with mutations in the nucleotide-binding oligomerization domain containing 2 (NOD2) gene. Biologic therapy of BS yielded diverse results. We aimed to evaluate clinical features and outcomes of Chinese patients with BS who were treated with tumor necrosis factor (TNF)α inhibitors.

Methods

A total of four patients with BS were diagnosed and treated with infliximab (IFX) at the Peking Union Medical College Hospital during 2015 to 2018 and were followed up for 18 months. All patients were systematically studied for treatment outcomes including the clinical manifestations and inflammatory markers. We also conducted a comprehensive literature review about TNFα inhibitor therapy in BS.

SUBMITTER: Chen J 

PROVIDER: S-EPMC6852754 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature