The role of telomeres in pulmonary fibrosis and its effects on choices of treatment
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Idiopathic pulmonary fibrosis (IPF) is an aggressive disease with no known origin. Scar tissue continues to build up in the lungs diminishing pulmonary function. Without successful treatment, patients with IPF have a mean survival of 5 years after diagnosis. Though the exact etiology of IPF is unknown, studies have established a role of telomeres in 25% or more of familial or sporadic cases. Mutations in the genes that encode for the components of telomerase have been implicated as the cause of the telomere dysfunction seen in these cases. The mutations examined below include TERT, TERC, RTEL1, TINF2, and PARN. With this link in mind, the available and the researched treatment plans are discussed. Of the discussed therapies, the researched treatment options that target the telomere-specific cases of IPF are a synthetic sex hormone (danazol), stem cell therapy, and a small molecule activator (GRN510). The remaining treatment plans discussed target either the direct cause of symptoms or the symptoms themselves. These studied options include N-acetylcysteine, prednisone, azathioprine, pirfenidone, nintedanib (with and without statins), and low-dose inhaled carbon monoxide. The treatment options with the favored positive results are danazol and nintedanib.
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