Idiopathic pulmonary fibrosis (IPF), is a disease of scarring of lungs which mostly affects old age and middle aged adults, without a known cause. Since the exact cause of Idiopathic pulmonary fibrosis (IPF) is still unknown, it is seen that genetics can play a role in being a staunch factor. When multiple members of the same family have idiopathic pulmonary fibrosis, the disease is also known as familial Idiopathic pulmonary fibrosis (IPF).

Idiopathic Pulmonary Fibrosis

Causes of Idiopathic Pulmonary Fibrosis

As stated above, the exact cause of idiopathic pulmonary fibrosis is still unknown there are certain conditions related to this condition:

  • Chronic inflammatory processes (sarcoidosis, Wegener granulomatosis)
  • Infection
  • Various environmental agents such as asbestos, silica and then exposure to certain gases
  • Exposure to certain radiation
  • Various chronic condition (lupus and rheumatoid arthritis)
  • Certain drugs and medication

Another related condition is hypersensitivity pneumonitis in which idiopathic pulmonary fibrosis can develop. Idiopathic pulmonary fibrosis (IPF) results from inhaling any dust contaminated with any bacteria, fungus or any type of animal product.

Also in some people, chronic pulmonary fibrosis develops without any known cause. Many people with Idiopathic pulmonary fibrosis (IPF) do not respond to the various medical therapies. Whereas in some patients with nonspecific interstitial pneumonitis (NSIP), respond to the immune suppressive therapy.

Stages of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis has been very loosely staged by medical experts and the disease varies from mild, moderate, severe or early advanced disease. Stages of idiopathic pulmonary fibrosis are obtained on the basis of pulmonary function test.

Here are two proposed staging systems as given:

  • Mortality Risk Scoring Systems - this is based on just four predictors:
    • Age
    • Any recent respiratory hospitalization
    • Based on the baseline forced vital capacity or FVC( a measure of the lung function)
    • 24 week changes in FVC
  • GAP Index - the system of staging in this disease is loosely based on the 4 baseline variables:
    • Gender
    • Age
    • Two lungs physiology variable (PV), (FVC including the diffusion capacity of the lung for carbon monoxide)
    • The index of GAP uses some of these variables which categorizes the patients in any three different stages which estimates the individual risk life of patients

Simply said, on the basis of various parameters, the stages of idiopathic pulmonary fibrosis are judged. Based on the pulmonary function test the stages are graded as

  • Mild – FVC more than 75%
  • Moderate – FVC between 50 to 75%
  • Severe – FVC between 25-49%
  • Very Severe – FVC less than 25%

Further stages of idiopathic pulmonary fibrosis also depended on the results of six minute walking test and other advanced investigations and scans.

Signs and Symptoms of Idiopathic Pulmonary Fibrosis

Usually the signs and symptoms of idiopathic pulmonary fibrosis have a very slow and relentless progression in the human body. Here are some signs and symptom of idiopathic pulmonary fibrosis:

  • In the earlier or the beginning stages, the patient suffering from Idiopathic pulmonary fibrosis (IPF) might complain of a dry persistent cough
  • Gradual and insidious onset of short breathlessness would set in
  • This shortness of breath condition worsens over time. Shortness of breath or dyspnea occurs with various activities are some of the common symptoms of idiopathic pulmonary fibrosis. Over the time, dyspnea occurs with less or no activity. Here, the shortness of breath becomes more disabling and it limits all the activity.
  • Only in rare cases, does fibrosis of lungs rapidly progresses and with the shortness of breath. In such cases, dyspnea and the disability occurs in weeks and months starting from the onsets of disease. This type and form of pulmonary fibrosis is also known as Hamman-Rich Syndrome.

Treatment of Idiopathic Pulmonary Fibrosis

Treatment of idiopathic pulmonary fibrosis usually depends on any related condition that can be treated. So far no effective or perfect treatment for Idiopathic pulmonary fibrosis (IPF) has been developed yet since scarring once developed is permanent. The treatment options for Idiopathic pulmonary fibrosis (IPF) are very limited.

A promising treatment of idiopathic pulmonary fibrosis is lung transplantation, the only therapeutic option available till now. And at times, lung diagnosis becomes difficult even when the tissue biopsy has been reviewed by the pathologists. However, research trials are being done so by using different sort of drugs, which help in reducing the ongoing scarring in the lungs.

Newer drugs are being researched and are tried, which may bring in some hope in the treatment of idiopathic pulmonary fibrosis. So far the results from such newer drugs obtained have also shown slow progression of scarring of lungs and it has also brought some minor changes in the lung tests done. But unfortunately, few of the side effects due to consumption of these medications have forced patients to cease their intake.

As a part of the treatment of lung fibrosis corticosteroids and other drugs that suppress the immune system of the body, may be prescribed to slow the progression of fibrosis. But the fact is these type of drugs do not help in the idiopathic pulmonary fibrosis. Similar palliative treatment and medications to control the symptoms may be prescribed.

It must also be borne in the mind that the toxic level and side effects of the medications can be very dangerous too. Some of the treatments used to treat such conditions include:

  • Cyclophosphamide
  • Azathioprine
  • Methotrexate
  • Penicillamine
  • Cyclosporine

Also, the anti-inflammatory medicine colchicine also is used; but with a very limited form of success. Other trials and tests done with other drugs such as gamma interferon and other immune suppressants are bring tried for treatment of idiopathic pulmonary fibrosis.

Pulmonary artery is a vessel that carries blood from the heart to the lungs to oxygenate it. Pulmonary fibrosis also reduces the oxygen levels in the blood, leading to hypoxia and can also elevate pressure in the pulmonary artery of the heart. The condition is pulmonary hypertension and can also lead to failure of the right ventricle of heart. Hence, patients suffering from pulmonary hypertension are also alongside treated with supplemental oxygen to prevent pulmonary hypertension.

Prognosis of Idiopathic Pulmonary Fibrosis

Prognosis of idiopathic pulmonary fibrosis depends on the stage of the disease and the overall health condition of the person. The prime goal of treatment for Idiopathic pulmonary fibrosis (IPF)by using the immune suppressing drugs like the corticosteroids is decreasing the inflammation of lungs and subsequent scarring in the body. Various responses to the treatments are seen. But once the scarring has developed, it becomes permanent. In many cases, Idiopathic pulmonary fibrosis (IPF) turns out to be a terminal illness and may show a life expectancy of around 3 to 5 years after diagnosis.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: August 24, 2017

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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