Rituximab and Abatacept Are Effective in Differential Treatment of Interstitial Lymphocytic Lung Disease in Children With Primary Immunodeficiencies.

ABSTRACT:

Background

Interstitial lymphocytic lung disease (ILLD), a recently recognized complication of primary immunodeficiencies (PID), is caused by immune dysregulation, abnormal bronchus-associated lymphoid tissue (BALT) hyperplasia, with subsequent progressive loss of pulmonary function. Various modes of standard immunosuppressive therapy for ILLD have been shown as only partially effective.

Objectives

To retrospectively evaluate the safety and efficacy of abatacept or rituximab in treatment of ILLD in children with PID.

Methods

29 children (median age 11 years) with various forms of PID received one of the two therapy regimens predominantly based on the lesions' immunohistopathology: children with prevalent B-cell lung infiltration received rituximab (n = 16), and those

SUBMITTER: Rodina Y 

PROVIDER: S-EPMC8458825 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature