Abstract
Pulmonary fibrosis is a disease characterized by progressive scarring of the lungs, with idiopathic pulmonary fibrosis (IPF) being the most aggressive form. The diagnosis of IPF is made after other conditions are excluded and is based on a characteristic clinical presentation, radiographic features and, sometimes, pathologic specimen. Existing IPF drug regimens, including corticosteroids and cytotoxic medications, are generally ineffective. To date, only lung transplantation has been shown to improve mortality in carefully selected patients. Multiple therapeutic agents have been investigated but none have proven to be successful. Novel drugs are constantly being sought in an attempt to find a therapy that halts or reverses this disease. Imatinib mesylate is used for chronic myelogenous leukemia and gastrointestinal stromal tumors. It also has antifibrotic properties, as demonstrated in several studies using mouse models of pulmonary fibrosis. Currently, trials are underway to investigate its efficacy in human subjects with IPF.
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