In Vivo and In Vitro Production of Interferon- γ in Pediatric Patients with Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a very rare and progressive disease in the pediatric patients. Immunologic mechanisms are involved in IPF pathogenesis. The theory that inflammation may arise from an imbalance between Th1 and Th2 cells has focused attention on the Th1-type cytokine interferon-γ (IFN-γ). We have determined levels of IFN-γ in the serum and in the leukocyte culture medium of 2 pediatric patients displaying different IPF disease duration and severity. Peripheral blood mononuclear cells of the 2 patients have shown decreased production of IFN-γ after mitogen stimulus. This event might indicate considering the feasibility of IFN-γ administration. We conclude that IFN-γ evaluations might be performed in cases of pediatric IPF to potentially suggest substitutive therapy with this cytokine.