Idiopathic Pulmonary Fibrosis - Treatment

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Key Points Î Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause occurring in adults. Î Radiologic and/or histopathologic patterns are consistent with usual interstitial pneumonia. Î Significant advances have been recently made in the clinical management of IPF. Î All of the recommendations in favor of treatment are conditional, meaning that decisions to treat patients with IPF should be individualized. Clinicians should be cautious to not interpret recommendations with the same grade as having the same net effects. Î The recommendations in favor of treatment put a higher value on the potential benefit of patient-important outcomes such as disease progression as measured by FVC decline and mortality and a lower value on potentially significant adverse effects and the cost of treatment. Treatment Table 1. Recommended Drugs a Brand Generic How Supplied Dose Esbriet ® Pirfenidone 267 mg capsules With food: Day 1-7: 1 capsule tid Day 8-14: 2 capsules tid Day 15 onward: 3 capsules tid OFEV ® Nintedanib 150 mg capsules 100 mg capsules With food: 150 mg capsule q12h (Temporary interruption or reduction to 100 mg bid for adverse effects) a Most of the evidence that provided the foundation for the suggestion to use regular antiacid therapy was derived from patients receiving proton pump inhibitor therapy. A very small proportion received histamine-2 receptor antagonists. Other antiacid treatments may need to be considered differently.

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