Management of Idiopathic Pulmonary Fibrosis
Section snippets
Pharmacologic treatments
The pharmacologic approach to IPF management has changed as the understanding of the pathogenesis of the disease has evolved over the last decade. The initial thinking was in favor of a disease triggered by a persistent inflammatory process, resulting in the induction of fibrosis and scarring of the lungs. As such, several trials were performed evaluating the efficacy of drugs that primarily exert their functions by suppressing inflammatory or immune responses (such as corticosteroids and
Management of comorbidities
IPF is often associated with morbidities (eg, PH and gastroesophageal reflux) and symptoms (eg, dyspnea, exercise limitation, fatigue, anxiety, mood disturbance, sleep disorders) that dramatically affect patients’ lives.
PH affects most patients with IPF at the time of initial diagnosis, and ultimately many of them during the course of the disease.29 Patients with IPF with concomitant PH (defined as a mean pulmonary artery pressure >25 mm Hg on right heart catheterization) have more dyspnea,
Management of acute exacerbation of IPF
The clinical course of IPF is usually chronic and slowly progressive, although some patients experience rapidly progressive disease.1 Acute worsening may occur as a consequence of multiple distinct causes, including respiratory infections, pulmonary embolism, pneumothorax, and heart failure; sometimes worsening cannot be linked to any identifiable cause, and this latter case is referred to as acute exacerbation of IPF (AE-IPF)43 (see article elsewhere in this issue). The prognosis of AE-IPF is
Nonpharmacologic treatments
Despite the lack of high-quality data demonstrating its benefit in patients with IPF, long-term oxygen therapy is commonly prescribed to patients showing resting hypoxemia or significant oxygen desaturation on exercise, and is strongly recommended by current guidelines.1 Due to the progressive nature of IPF, higher flow rates than those commonly used in chronic obstructive pulmonary disease are likely to be required. Supplemental oxygen may improve symptoms, quality of life,52 and endurance
Lung transplantation
Pulmonary fibrosis represents the second most frequent disease for which lung transplantation is performed.64 More recently the number of lung transplants performed for IPF has steadily increased, particularly in the United States, where IPF now represents the leading indication for lung transplantation.65 Five-year survival rates after lung transplantation in IPF are estimated at 50% to 56%.62, 66, 67 Additional evidence suggests that patients with pulmonary fibrosis undergoing lung
Summary
Despite more than a decade of efforts to show a definite effect on disease course in IPF, a treatment regimen that is unanimously recognized as a standard of care is still lacking. Current guidelines recommend enrollment in clinical trials as the standard of care for patients with IPF. However, given that one drug, pirfenidone, is currently approved for the treatment of IPF in parts of the world that are home to about 2 billion people, it is reasonable to say that a major step forward has been
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Cited by (9)
Emerging targets for the treatment of idiopathic pulmonary fibrosis
2013, Annual Reports in Medicinal ChemistryCitation Excerpt :Recommended therapeutic regimens for the treatment of IPF continue to evolve, with evidence-based treatments varying from patient to patient. The currently preferred treatment options for acute exacerbations in IPF include limited pharmacological-based therapies (corticosteroids) and non-pharmacological-based approaches (long-term oxygen therapy and lung transplantation).11 The strategy for developing pharmacological therapies that are specifically aimed at attenuating the fibrotic response gained acceptance, while the development of anti-inflammatory approaches has fallen out of favor in the last decade.
Modulation of Bleomycin-induced Oxidative Stress and Pulmonary Fibrosis by Ginkgetin in Mice via AMPK
2023, Current Molecular PharmacologyRecent advances in managing idiopathic pulmonary fibrosis
2017, F1000ResearchIdiopathic pulmonary fibrosis: Current treatment options and critical appraisal of nintedanib
2015, Drug Design, Development and TherapyChallenges in idiopathic interstitial lung disease
2015, Minerva Pneumologica
Financial disclosures: S.C., P.S. and F.L. have nothing to disclose; L.R. reports receiving consulting fees from Boehringer Ingelheim, Intermune, Celgene and Gilead and lecture fees from Intermune.