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

Granulomatous-Lymphocytic Interstitial Lung disease (GLILD) is a granulomatous and lymphoproliferative condition occurring in ~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...

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Автори: Robert Tillman (Автор), R. Paul Guillerman (Автор), Timothy Trojan (Автор), Manuel Silva-Carmona (Автор), Ivan K. Chinn (Автор)
Формат: Книга
Опубліковано: Frontiers Media S.A., 2019-03-01T00:00:00Z.
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100 1 0 |a Robert Tillman  |e author 
700 1 0 |a R. Paul Guillerman  |e author 
700 1 0 |a Timothy Trojan  |e author 
700 1 0 |a Manuel Silva-Carmona  |e author 
700 1 0 |a Manuel Silva-Carmona  |e author 
700 1 0 |a Ivan K. Chinn  |e author 
245 0 0 |a Treatment-Responsive Granulomatous-Lymphocytic Interstitial Lung Disease in a Pediatric Case of Common Variable Immunodeficiency 
260 |b Frontiers Media S.A.,   |c 2019-03-01T00:00:00Z. 
500 |a 2296-2360 
500 |a 10.3389/fped.2019.00105 
520 |a Granulomatous-Lymphocytic Interstitial Lung disease (GLILD) is a granulomatous and lymphoproliferative condition occurring in ~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. 
546 |a EN 
690 |a Granulomatous-Lymphocytic Interstitial Lung disease 
690 |a Common Variable Immunodeficiency 
690 |a chemotherapeutic agents 
690 |a pediatrics 
690 |a treatment 
690 |a Pediatrics 
690 |a RJ1-570 
655 7 |a article  |2 local 
786 0 |n Frontiers in Pediatrics, Vol 7 (2019) 
787 0 |n https://www.frontiersin.org/article/10.3389/fped.2019.00105/full 
787 0 |n https://doaj.org/toc/2296-2360 
856 4 1 |u https://doaj.org/article/3fdbcedf32b44775bfa3224f4ea509f7  |z Connect to this object online.