Interferon gamma-1b as Therapy for Idiopathic Pulmonary Fibrosis

Abstract

Background: The only proven therapeutic option for idiopathic pulmonary fibrosis (IPF) is lung transplantation. It is biologically plausible that interferon gamma-1b (G-IFN) may halt or even reverse the disease process and therefore prove to be an effective medical therapy. We report our results using this medication in a cohort of patients with a wide range of severity of IPF. Objectives: To determine the impact of G-IFN therapy on the progression of disease in patients with IPF. Methods: We performed a retrospective analysis of patients’ pulmonary function tests (PFTs), specifically the forced vital capacity (FVC) and the single breath diffusing capacity for carbon dioxide (DL_CO). Comparisons of these parameters prior to and after the implementation of therapy were made by generating regression slopes by least-squares equations. Results: Twenty-two patients qualified for the analysis. For the FVC, comparison of pre- and post-therapy best-fit regression lines demonstrated a significant difference in favor of G-IFN (p < 0.015). For the DL_CO, a significant difference in favor of gamma interferon therapy was detected in the advanced group (p < 0.03). Seventy percent of the patients showed either stabilization or regression of disease in both their FVCs and their DL_CO. Conclusion: G-IFN appears to be an effective new therapy for patients with IPF. Salutary effects on the rate of change in the FVC and DL_CO were most apparent in patients with advanced disease. With slowing or reversal in loss of lung function, G-IFN may improve longevity and may have utility as a bridge to lung transplantation in those patients who are appropriate candidates.

Long-term clinical effects of interferon gamma-1b and colchicine in idiopathic pulmonary fibrosis

“…Similarly, PRASSE et al [36] found improvement in physiological function in only one of five patients. In contrast, NATHAN et al [35] reported that, paradoxically, patients with advanced disease appear to derive the most benefit from IFN-c therapy.…”

“…Two small retrospective noncomparative [34,35] and one prospective noncomparative study [36] of IFN-c therapy for IPF have shown controversial results regarding the usefulness of IFN-c therapy. KALRA et al [34] observed symptomatic and functional improvement in only one out of the 21 patients treated.…”

Interferon: Cellular Executioner or White Knight?

“…IFN-γ1b (ACTIMMUNE), as an antifibrotic agent has been used, with varying degrees of success, in the treatment of idiopathic pulmonary fibrosis [138,139], as well as ovarian cancer when used in combination with platinum-based chemotherapies [140][141][142], bladder carcinoma [143], systemic sclerosis/scleroderma [144][145][146], and tuberculosis [147][148][149][150]. See Table 3 for a summary of IFNs currently available for therapy or in clinical trials.…”

Lung Remodeling in Interstitial Pneumonia: A New Molecular Target of Pulmonary Fibrosis

“…In 1999, Ziesche R et al reported that among patients with usual interstitial pneumonia, those who received interferon gamma-1b and prednisolone showed larger total lung capacity, and better partial pressure of arterial oxygen (PaO2), than those who received only prednisolone [48]. A retrospective analysis of a small population with IPF revealed salutary effects on the rate of change in the FVC and DLCO, especially apparent in patients with advanced disease [49]. But in 2004, a doubleblind, multi national trial revealed that there is no significant difference in progression-free survival, pulmonary function, or the quality of life [50].…”