Autoantibody-Targeted Treatments for Acute Exacerbations of Idiopathic Pulmonary Fibrosis

摘要: Background Severe acute exacerbations (AE) of idiopathic pulmonary fibrosis (IPF) are medically untreatable and often fatal within days. Recent evidence suggests autoantibodies may be involved in IPF progression. Autoantibody-mediated lung diseases typically refractory to glucocorticoids nonspecific medications, but frequently respond focused autoantibody reduction treatments. We conducted a pilot trial test the hypothesis that autoantibody-targeted therapies also benefit AE-IPF patients. Methods Eleven (11) critically-ill patients with no conventional autoimmune were treated therapeutic plasma exchanges (TPE) rituximab, supplemented later cases intravenous immunoglobulin (IVIG). Plasma anti-epithelial (HEp-2) matrix metalloproteinase-7 (MMP7) evaluated by indirect immunofluorescence ELISA, respectively. Outcomes among subjects compared those 20 historical control glucocorticoid therapy prior this experimental trial. Results Nine (9) (82%) had improvements gas exchange after treatment, one (5%) control. Two three who relapsed only five TPE responded again additional TPE. The latest an augmented regimen nine plus rituximab IVIG have sustained responses without relapses 96-to-237 Anti-HEp-2 present therapy, reduced treatment. Conversely, MMP7 levels not systematically affected nor correlated clinical responses. One-year survival was 46+15% vs. 0% controls. No serious adverse events attributable medications. Conclusion This indicates specific treatments reduce might some severely-ill patients. These findings potential implications regarding mechanisms progression, justify considerations for incremental trials patients. Trial Registration ClinicalTrials.gov NCT01266317