Management of Idiopathic Pulmonary Fibrosis

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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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    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.

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