Management of Idiopathic Pulmonary Fibrosis

Ann Pharmacother. 2019 Dec;53(12):1238-1248. doi: 10.1177/1060028019862497. Epub 2019 Jul 7.

Abstract

Objective: Provide information for pharmacists on idiopathic pulmonary fibrosis (IPF) and its treatment. Study Selection and Data Extraction: All articles with data from randomized controlled trials of nintedanib or pirfenidone were reviewed. Data Synthesis: IPF is a progressive and ultimately fatal interstitial lung disease characterized by decline in lung function and worsening dyspnea. It is uncommon and mainly occurs in individuals aged >60 years, particularly men with a history of smoking. Nintedanib and pirfenidone were approved in the United States for the treatment of IPF in 2014 and received conditional recommendations in the 2015 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association treatment guidelines. These drugs slow the progression of IPF by reducing the rate of decline in lung function. Their adverse event profile is characterized mainly by gastrointestinal events, which can be managed through dose adjustment and symptom management. Management of IPF should also include smoking cessation, vaccinations, and supportive care such as patient education, pulmonary rehabilitation, and the use of supplemental oxygen as well as optimizing the management of comorbidities. Relevance to Patient Care and Clinical Practice: This review provides clinical pharmacists with information on the course of IPF, what can be expected of current treatments, and how to help patients manage their drug therapy. Conclusions: IPF is a progressive disease, but treatments are available that can slow the progression of the disease. Clinical pharmacists can play an important role in the care of patients with IPF through patient education, monitoring medication compliance and safety, ensuring drugs for comorbidities are optimized, and preventive strategies such as immunizations.

Keywords: disease management; drug information; drug trials; interstitial lung disease; patient education.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Comorbidity
  • Disease Progression
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Indoles / administration & dosage
  • Indoles / adverse effects
  • Indoles / therapeutic use*
  • Male
  • Medication Adherence
  • Middle Aged
  • Patient Education as Topic / organization & administration
  • Pharmacists / organization & administration*
  • Pyridones / administration & dosage
  • Pyridones / adverse effects
  • Pyridones / therapeutic use*

Substances

  • Indoles
  • Pyridones
  • pirfenidone
  • nintedanib