Patients &

Care Partners

 

PF is Scarring of the Lungs

Years to Diagnosis

1.5

Affected in Kansas and Missouri

>26,000

>200

Types of Pulmonary Fibrosis

ILD Patients Across the US

>500,000

>50,000

Diagnoses per Year in the US

Americas Die from IPF Each Year

>40,000

Misdiagnoses before Correct Diagnosis

3

Information obtained from the Pulmonary Fibrosis Foundation 3.30.2018

 

Pulmonary Fibrosis

Pulmonary Fibrosis (PF) kills the same number of people each year as many forms of cancers. The odds of surviving for 5 years was much worse before the development of new anti-fibrotic medications that became available in the last 5 years. Unfortunately, these drugs do not offer a cure, nor are they as effective in advanced disease. Early recognition of symptoms is essential to early diagnosis. Shortness of breath on stairs/inclines, or a chronic dry cough needs to be checked out. Do not dismiss these symptoms as allergies or a normal part of aging! Most people with PF experience chronic cough or mild shortness of breath for 1 to 2 years before being diagnosed.

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Pulmonary Fibrosis can cause progressive scarring of the lungs. The worst form of PF, called Idiopathic Pulmonary Fibrosis (IPF), had an average survival of 27 months after diagnosis before the first two anti-scarring medications were FDA-approved in 2014. These two medications Esbriet and Ofev slow the progression of the disease but are not a cure. There are new and potentially more effective treatments being tested in clinical trials, but early detection is still key. Because X-rays can miss early disease, PF is best detected by a CT scan.

There are different causes of pulmonary fibrosis. Autoimmune disease, exposure to occupational or environmental hazards, taking certain drugs, and radiation exposure are some of the more common causes. Genetics also play a role in pulmonary fibrosis: anywhere from 3 – 20% of patients have another family member with PF.

Interstitial Lung Disease (ILD) Classifications

Hypersensitivity Pneumonitis (HP)

Idiopathic Interstitial Pneumonias (IIPs)

Autoimmune ILD

Other ILDs

  • Rheumatoid Arthritis ILD 

  • Sjoren's Syndrome ILD

  • Systemic Lupus Erythematous 

  • Polymyositis and Dermatomyositis

  • Mixed Connective Tissue ILD

  • Systemic Sclerosis ILD

  • Other Connective Tissue ILDs

  • Idiopathic Lymphoid Interstitial Pneumonia

  • Idiopathic Pleuroparenchymal Fibroelastosis

  • Unclassified   Idiopathic Interstitial Pneumonais

  • Idiopathic Pulmonary Fibrosis (IPF)

  • Idiopathic Non-Specific Interstitial Pneumonia 

  • Respiratory Bronchiolitis-ILD

  • Desquamative  Interstitial Peumonia

  • Cyptogenic Organizing Pneumonia

  • Acute Interstitial Pneumonia

The umbrella of Interstitial Lung Disease (ILD) includes over 200 different diseases and one of the ways ILD can be classified is listed above.  This makes a correct diagnosis extremely important because not all ILD is treated the same.  

  • Sacroidosis

  • Lymphangioleio-myomatosis (LAM)

  • Langerhans Cell Histiocytosis (LCH)

  • Drug-Associated ILD

  • Other Exposure ILDs

  • Vasculitis/ Granulomatosis ILDs

  • Eosinophilic Pneumonias

  • Alveolar Proteinosis

  • Other Rare ILDs

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© 2019 The Kansas City Foundation for Pulmonary Fibrosis.  All rights reserved