Idiopathic Pulmonary Fibrosis (IPF) Questions & Answers

Updated: Jul 15, 2019
  • Author: Amanda M K Godfrey, MD; Chief Editor: Guy W Soo Hoo, MD, MPH  more...
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Questions & Answers

Overview

What is idiopathic pulmonary fibrosis?

What are the symptoms of idiopathic pulmonary fibrosis?

What are the systemic symptoms of idiopathic pulmonary fibrosis?

How is idiopathic pulmonary fibrosis diagnosed?

What are the physical exam findings in idiopathic pulmonary fibrosis?

Which lab tests are used to diagnose idiopathic pulmonary fibrosis?

Which imaging studies are used to diagnose idiopathic pulmonary fibrosis?

Which procedures are used to diagnose idiopathic pulmonary fibrosis?

How is idiopathic pulmonary fibrosis treated?

How is surgery used to treat idiopathic pulmonary fibrosis?

How is drug treatment used for idiopathic pulmonary fibrosis?

What is idiopathic pulmonary fibrosis (IPF)?

What is the relevant background information on idiopathic pulmonary fibrosis (IPF)?

What is the pathophysiology of idiopathic pulmonary fibrosis (IPF)?

What is the role of genetics in the pathogenesis of idiopathic pulmonary fibrosis (IPF)?

What causes idiopathic pulmonary fibrosis (IPF)?

What is the role of genetic mutations in the etiology of idiopathic pulmonary fibrosis (IPF)?

How common is idiopathic pulmonary fibrosis (IPF) in the US?

What is the global incidence of idiopathic pulmonary fibrosis (IPF)?

What are the race-related demographics of idiopathic pulmonary fibrosis (IPF)?

Is idiopathic pulmonary fibrosis (IPF) more common in men or women?

What are the age-related demographics of idiopathic pulmonary fibrosis (IPF)?

What is the mortality rate of idiopathic pulmonary fibrosis (IPF)?

What is the prognosis of idiopathic pulmonary fibrosis (IPF)?

What educational information is available for patients with idiopathic pulmonary fibrosis (IPF)?

Presentation

What are the history findings of idiopathic pulmonary fibrosis (IPF)?

What are common physical exam findings in idiopathic pulmonary fibrosis (IPF)?

What are the complications of idiopathic pulmonary fibrosis (IPF)?

DDX

What are the differential diagnoses for Idiopathic Pulmonary Fibrosis (IPF)?

Workup

Which lab studies are indicated in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of chest radiography in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of high-resolution CT scanning in the workup of idiopathic pulmonary fibrosis (IPF)?

What are the four diagnostic categories recommended for use in interpreting HRCT patterns in IPF diagnosis?

What is the role of pulmonary function testing in the workup of idiopathic pulmonary fibrosis (IPF)?

How is the 6-minute walk test used in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of bronchoalveolar lavage (BAL) in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of transthoracic echocardiography in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of bronchoscopy in the workup of idiopathic pulmonary fibrosis (IPF)?

What is the role of lung biopsy in the workup of idiopathic pulmonary fibrosis (IPF)?

What are the diagnostic criteria for idiopathic pulmonary fibrosis (IPF)?

What are the histopathological findings in idiopathic pulmonary fibrosis (IPF)?

What is the significance of fibroblastic foci in the histologic findings in idiopathic pulmonary fibrosis (IPF)?

How is honeycomb change characterized in the histologic findings of idiopathic pulmonary fibrosis (IPF)?

What are the histopathological criteria for UIP pattern in the workup of idiopathic pulmonary fibrosis (IPF)?

Treatment

What is the role of managing comorbidities in the treatment of idiopathic pulmonary fibrosis (IPF)?

What other interventions are helpful for the treatment of idiopathic pulmonary fibrosis (IPF)?

How is idiopathic pulmonary fibrosis (IPF) surgically treated?

What is acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF)?

What are the diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF)?

What is the inpatient care of idiopathic pulmonary fibrosis (IPF)?

When is transfer indicated in the treatment of idiopathic pulmonary fibrosis (IPF)?

Which specialist consultations are indicated in the treatment of idiopathic pulmonary fibrosis (IPF)?

What dietary recommendations are indicated in the treatment of idiopathic pulmonary fibrosis (IPF)?

What activity recommendations are indicated in the treatment of idiopathic pulmonary fibrosis (IPF)?

How are patients with idiopathic pulmonary fibrosis (IPF) monitored on a long-term basis?

Guidelines

What are the guidelines on idiopathic pulmonary fibrosis (IPF) by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association?

Medications

What is the role of interferon-Y1b in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of anti-inflammatory agents and immune modulators in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the approach to medical therapy for the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of corticosteroids in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role N -acetylcysteine (NAC) in the treatment of idiopathic pulmonary fibrosis (IPF)?

What were the findings of the PANTHER-IPF (idiopathic pulmonary fibrosis) trial?

What are the precautions of using N-acetylcysteine monotherapy in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of etanercept in the treatment of idiopathic pulmonary fibrosis (IPF)?

How is bosentan used to treat idiopathic pulmonary fibrosis (IPF)?

What is the role of sildenafil in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of nintedanib (Ofev) in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of pirfenidone (Esbriet) in the treatment of idiopathic pulmonary fibrosis (IPF)?

What is the role of colchicine in the treatment of idiopathic pulmonary fibrosis (IPF)?

Which medications in the drug class Immunosuppressant Agent are used in the treatment of Idiopathic Pulmonary Fibrosis (IPF)?

Which medications in the drug class Corticosteroid, Systemic are used in the treatment of Idiopathic Pulmonary Fibrosis (IPF)?

Which medications in the drug class Antifibrotic Agents are used in the treatment of Idiopathic Pulmonary Fibrosis (IPF)?

Which medications in the drug class Tyrosine Kinase Inhibitors are used in the treatment of Idiopathic Pulmonary Fibrosis (IPF)?