CLINICAL REVIEW / PEER REVIEWED

Pharmacologic Treatments for Idiopathic Pulmonary Fibrosis

Clinician Reviews. 2018 April;28(4):16-21

References

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14. Chowdhury BA; FDA. Two FDA drug approvals for idiopathic pulmonary fibrosis (IPF). October 15, 2014. https://blogs.fda.gov/fdavoice/index.php/2014/10/two-fda-drug-approvals-for-idiopathic-pulmonary-fibrosis-ipf/. Accessed March 12, 2018.
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Patient monitoring

The ATS recommends measuring FVC and DL_CO every three to six months, or sooner if clinically indicated.¹³ Pulse oximetry should be measured at rest and on exertion in all patients, regardless of symptoms, to assure proper saturation and identify the need for supplemental oxygen; this should also be done every three to six months.

The ATS recommends prompt detection and treatment of comorbidities such as pulmonary hypertension, emphysema, airflow obstruction, GERD, sleep apnea, and coronary artery disease.¹³ These recommendations are based on the organization’s 2015 guidelines.

OUTCOME FOR THE CASE PATIENT

The patient was started on pirfenidone (2,403 mg/d). He is continuing treatment and showing improvements in quality of life and slowed deterioration of lung function.

CONCLUSION

IPF causes progressive fibrosis of lung interstitium. The etiology is unknown, the symptoms and signs are vague, and mean life expectancy following diagnosis is two to five years. The most recent IPF guidelines recommend avoiding use of anticoagulants and immunosuppressants (eg, steroids, azathioprine, and N-acetylcysteine), due to their proven ineffectiveness and harm to patients with IPF.

Since the FDA’s approval of pirfenidone and nintedanib, the ATS has made recommendations for their use in patients with IPF. Despite mixed results in clinical trials, both drugs have demonstrated the ability to slow the decline in FVC over time, with relatively benign adverse effects. It is difficult to compare pirfenidone and nintedanib, or to recommend use of one drug over the other. However, it is promising that patients with this routinely fatal disease now have treatment options that can potentially modulate their disease progression.

Pharmacologic Treatments for Idiopathic Pulmonary Fibrosis

References

Patient monitoring

OUTCOME FOR THE CASE PATIENT

CONCLUSION

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