Skill Checkup: A 63-Year-Old Man With Persistent Cough and New Shortness of Breath

Zab Mosenifar, MD


January 27, 2023

In patients with IPF, treatment guidelines recommend nintedanib, which targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and platelet-derived growth factor (PDGF) receptors. Compared with more selective tyrosine kinase inhibitors, nintedanib appears to have benefit in terms of patient-reported outcomes, including improvement in outcomes such as disease progression as measured by rate of forced vital capacity decline. A pooled analysis of three trials looking at death from any cause over 52 weeks after treatment with nintedanib versus placebo did not show a statistically significant difference (hazard ratio, 0.70 [95% CI, 0.47-1.03; moderate confidence]; P = 0.14). Guidelines also make a recommendation for pirfenidone, an oral antifibrotic drug with pleiotropic effects. Pooled trial results have suggested improved mortality with its use.

The guidelines also advise against the use of these treatment strategies for IPF: anticoagulation (warfarin); imatinib, a selective tyrosine kinase inhibitor against PDGF receptors; combination prednisone, azathioprine, and N-acetylcysteine; and selective endothelin receptor antagonist (ambrisentan).


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