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Idiopathic Pulmonary Fibrosis

Abstract

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NORD is very grateful to David A. Schwartz, MD, Department of Medicine and Department of Immunology, University of Colorado School of Medicine, for assistance in the preparation of this report.

Synonyms of Idiopathic Pulmonary Fibrosis

  • cryptogenic fibrosing alveolitis
  • idiopathic diffuse interstitial pulmonary fibrosis
  • IPF

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disorder characterized by thickening, stiffening and scarring (fibrosis) of tissue within the lungs. Affected individuals develop shortness of breath and progressive lung disease. Ultimately, IPF results in life-threatening complications such as respiratory failure. The rate of progression can vary greatly from one person to another. Over years, most individuals experience increasing respiratory symptoms, progressive scarring of the lungs and a gradual decline in lung function. Less often, affected individuals have mild scarring within the lungs and little to no change in the disease for many years. In some cases, the disorder can progress rapidly (acutely), causing life-threatening complications within several years of diagnosis. The term ‘idiopathic’ means that the underlying cause of the disorder is unknown or unproven. Although there is no cure for IPF, various different treatments are available to manage the disorder and several newer therapeutic options are being studied. Ultimately, some affected individuals will require a lung transplant.

Introduction
IPF is classified as a form of idiopathic interstitial pneumonia, which is a group of lung diseases that damage the lungs in a similar manner and occur due to unknown causes. This group of disorders is also known as diffuse parenchymal lung diseases. Collectively, these disorders are classified under the broader umbrella term, interstitial lung diseases (ILDs). ILDs a large group of disorders (more than 200) characterized by progressive scarring of the lungs. IPF is the most common form.

Organizations related to Idiopathic Pulmonary Fibrosis

NORD offers an online community for this rare disease. RareConnect was created by EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders) to provide a safe space where individuals and families affected by rare diseases can connect with each other, share vital experiences, and find helpful information and resources. You can view these international, rare disease communities at www.rareconnect.org.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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