Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a rare disorder that affects your lungs and makes even daily activities impossible. Find out all about this condition, including its various causes, symptoms, treatment options and prognosis.

What is Idiopathic Pulmonary Fibrosis?

It is a chronic and progressive form of lung disease which results in scarring of lungs. This is an interstitial lung condition and is one of 200 disorders that give rise to similar symptoms although having varying causes. The scarring of the lungs generally initiates at the edges and then progressively proceeds towards the core of the lungs. This makes it more and more difficult to breathe for an affected person. Over time, the development of fibrous scar tissues in the lungs significantly affects the oxygen supply of the body. This makes IPF a disabling health condition that may have fatal consequences.

Idiopathic Pulmonary Fibrosis Alternate Names

IPF is known by several alternate names in the medical world. Some of these are:

  • Hamman-Rich syndrome
  • Chronic interstitial pneumonitis
  • Idiopathic diffuse interstitial pulmonary fibrosis
  • Pulmonary fibrosis
  • Fibrosing alveolitis
  • Diffuse fibrosing alveolitis
  • Cryptogenic fibrosing alveolitis or CFA
  • Usual interstitial pneumonitis or UIP

Idiopathic Pulmonary Fibrosis Causes

The epidemiology and pathogenesis of the condition has not been completely understood as yet. The term “Idiopathic” itself refers to the fact that no proper cause for this disease has yet been found. As of now, however, the disease is believed to be an abnormal inflammatory and fibrotic response to a microscopic injury that ultimately leads to pathological scarring. Apart from this, several genetic and epidemiological factors are also held responsible for this condition. If there is any causal relationship between these factors and IPF, the doctors would not diagnose the health condition as IPF. However, this is highly uncommon. The most common factors believed to be responsible for this disease are:

 

Picture 1 – Idiopathic Pulmonary Fibrosis

  • A long history of smoking
  • Gastroesophageal reflux disease (GERD)
  • Bacterial or viral lung infections
  • Prolonged exposure to dusts or environmental contaminants. Inorganic dusts such as silica, asbestos, beryllium and metal dusts as well as organic dusts like animal proteins and bacteria are proved to be critical.
  • Certain medicines like antibiotics (Sulfasalazine, Nitrofurantoin), anticonvulsants (Phenytoin), antiarrythmics (Propranolol, Amiodarone), chemotherapeutic agents (Bleomycin, Erbital, Methotrexate, Oxaliplatin).
  • Therapeutic radiation

IPF is also frequently linked with upper respiratory disorders, such as tuberculosis and pneumonia. The exact connection between IPF and these diseases is not known.

Interstitial lung diseases or ILD are also linked to connective tissue diseases such as lupus, scleroderma, rheumatoid arthritis and sarcoidosis.

Age is also supposed to be a causative factor for this condition. Most people affected by IPF are older and aged between 50 and 70.

Idiopathic Pulmonary Fibrosis Symptoms

The symptoms of IPF may not be evident at the onset of the disease and may only be apparent when the disease has progressed to an advanced level. When the health condition progresses and the lung damage become more severe, patients suffer from breathlessness even while doing simple physical activities like showering or getting dressed. In later stages, conversing with others and eating food become increasingly difficult.

Some of the most notable symptoms of this disorder are:

  • Dyspnea or shortness of breath
  • Occasional chest pains
  • Chronic dry cough
  • Reduced tolerance for activity
  • Shortness of breath while performing simple tasks
  • Loss of appetite
  • Weakness and fatigue
  • Discomfort and pain in the chest
  • Rapid weight loss
  • Clubbing or rounding of fingernails
  • Crackling sounds heard from the lungs
  • Cyanosis
  • Numbness in fingertips

Idiopathic Pulmonary Fibrosis Prevention

People with a prolonged history of smoking are mostly found to be suffering from IPF. Individuals exposed to high levels of industrial smoke and dust are also significantly affected by this disorder. Hence, avoiding smoking or dusty environments can help prevent this disease to a certain degree. However, a total prevention is difficult as the exact causes responsible for this health condition are unknown. A rare type of IPF is also known to affect individuals genetically.

Idiopathic Pulmonary Fibrosis Diagnosis

Physicians perform a variety of tests and adopt different diagnostic strategies to determine whether or not a patient has IPF. Firstly, doctors check the medical history of a patient thoroughly. Then they ask questions to find out if the suffering individual is or has been regularly exposed to an environment of industrial dusts. Apart from checking the environmental factors, doctors also investigate familial, occupational as well as other medical conditions which may have contributed to the disease. A physical exam of the lungs with a stethoscope may reveal crackling, Velcro-like sounds coming from the lungs. These sounds are made by small openings during the incoming of air. Other symptoms, such as clubbing of fingertips, can also confirm the presence of IPF. Some of the various medical tests and examinations carried out by doctors to diagnose this condition involve:

Chest X-Ray

An X-Ray examination of the chest is carried out as a screening process. However, the results of this test alone are not conclusive enough to indicate that the patient is suffering from IPF. Hence, additional tests are carried out to determine such results.

High Resolution Computerized Tomography (HRCT)

This test provides doctors with sharper and precise images of the lungs of a patient. It assists them in identifying certain specific clinical patterns which indicate that the patient is having IPF.

Pulmonary Function Tests

The aim of conducting these breathing examinations is to check whether the lungs can exchange carbon dioxide and oxygen properly.

Pulse Oximeter Test

It helps determine the amount of oxygen present in the bloodstream.

Arterial Blood Gas (ABG)

It is a medical examination that helps directly measure oxygen, carbon dioxide and arterial pH through an arterial puncture.

Bronchoscopy

Bronchoscopy helps in examining the main air passages of the lungs with the help of a flexible tube called bronchoscope.

Bronchoalveolar lavage (BAL)

It is performed with the aid of a bronchoscope. It is an easy way to collect cell samples from lower respiratory tract.

Surgical Lung Biopsy

It is believed to be the most potent tool to evaluate patients considered to be suffering from IPF. Hence, this test is considered to be the “Gold Standard”.

Exercise Testing

This is a 6-minute walk test (6MWT) conducted to determine the response of the lungs to exertion. Although the methods for conducting this test may vary from one institution to another, a treadmill or a stationary bike is generally employed. Blood pressure, oxygen saturation levels and EKG are monitored during this exercise test.

Histology/Pathology of Idiopathic Pulmonary Fibrosis

The histological specimens used to diagnose IPF should be large enough so that pathologists are easily able to detect the essential lung architecture. Small biopsies, such as the ones obtained through transbronchial lung biopsy during bronchoscopy, are generally considered to be insufficient for diagnosis of IPF. Hence, it is essential to obtain larger biopsies through Thoracoscopy or Thoracotomy.

The histological appearance that is closely associated with this disease is commonly referred to as UIP (Usual Interstitial Pneumonia). Although a diagnosis of UIP frequently corresponds to a diagnosis of IPF, the resemblance can be seen in many other disorders as well. The essential features of UIP include:

  • Interstitial fibrosis in “patchwork pattern”
  • Honeycomb changes
  • Interstitial scarring
  • Fibroblast foci

There are very little traces of interstitial chronic inflammation.

Idiopathic Pulmonary Fibrosis Treatment

At present, there are no known cures for this disorder. Since no consensus has yet been reached on what should be the ideal method of treatment for IPF, the treatment methods mentioned here should not be taken as a standard by everyone. This is due to the fact that the method of treatment varies from one patient to another and entirely depends on the medical practitioner handling a case of IPF.

  • Medications, such as cytotoxic drugs and corticosteroids, can reduce inflammation in some patients.
  • Oxygen supply is required to treat patients having lower oxygen levels in their bloodstream.
  • Pulmonary rehabilitation might not totally cure the disorder; however this procedure may help the patient to exercise without experiencing any breathing difficulties.
  • Lung transplant might be required to treat patients with advanced stages of IPF.
  • Presently, clinical studies are being conducted to discover new treatment methods for curing patients with IPF.

Idiopathic Pulmonary Fibrosis Prognosis

The condition has a poor prognosis. Certain patients show some signs of improvement when they are given cytotoxic drugs or corticosteroids. In most cases, however, the disease grows worse even with medical care. The worsening of this condition can happen slowly or quickly. A lot of IPF patients need supplementary oxygen for sustenance and some may require a lung transplant. This is due to the fact that IPF patients have very low life expectancy. Most individuals with IPF die within 3 to 5 years from the time when they are first diagnosed with the disease.

Idiopathic Pulmonary Fibrosis Complications

A person with IPF may suffer from any of the following complications:

 

Picture 2 – Idiopathic Pulmonary Fibrosis Image

  • Cor pulmonale
  • Chronic hypoxemia
  • Polycythemia
  • Pulmonary hypertension
  • Pneumothorax
  • Respiratory failure

Idiopathic Pulmonary Fibrosis Foundation

The Idiopathic Pulmonary Fibrosis Foundation is a nonprofit organization, the aim of which is to spread awareness of the disease, find a proper cure, stand for the community of pulmonary fibrosis and establish an environment of compassion and cooperation for the patients and their loved ones.

Idiopathic pulmonary fibrosis is a chronic lung disorder that can often prove to be fatal. Hence, it is necessary to provide patients of this disorder with timely care and attention to keep its symptoms of under control.