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Case Report ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Pediatr. | doi: 10.3389/fped.2019.00105

Treatment-Responsive Granulomatous-Lymphocytic Interstitial Lung Disease in a Pediatric Case of Common Variable Immunodeficiency

 Robert H. Tillman1, 2*,  R. P. Guillerman1, 2,  Timothy Trojan3, Manuel Silva-Carmona1, 2 and  Ivan K. Chinn1, 2
  • 1Baylor College of Medicine, United States
  • 2Texas Children's Hospital, United States
  • 3Allergy Partners of Oklahoma, United States

Granulomatous-Lymphocytic Interstitial Lung disease (GLILD) is a granulomatous and lymphoproliferative condition occurring in approximately 25% of Common Variable Immunodeficiency (CVID) patients with the highest prevalence in the late teen to young adult years. GLILD was first described in adults and carries a poor prognosis with survival estimated to be reduced by half. Here we report a pediatric case of CVID-associated GLILD that presented with rapid deterioration over 3 months and responded to adult-based treatment with dual chemotherapeutic agents (rituximab and azathioprine), resulting in complete resolution of clinical findings and near complete resolution of radiologic findings. This case highlights the opportunity to achieve a favorable outcome in GLILD following appropriate diagnosis and therapy.

Keywords: GLILD, CVID, pediatric, Chemotherapeutic agents, rituximab, Azathioprine, Treatment

Received: 14 Nov 2018; Accepted: 05 Mar 2019.

Edited by:

Rosa Bacchetta, Stanford University, United States

Reviewed by:

Oskar A. Haas, St. Anna Children’s Cancer Research Institute (CCRI), Austria
Michael D. Keller, Children’s National Health System, United States  

Copyright: © 2019 Tillman, Guillerman, Trojan, Silva-Carmona and Chinn. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD. Robert H. Tillman, Baylor College of Medicine, Houston, United States,