Idiopathic Pulmonary Fibrosis

What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) is a progressive chronic disease that damages the air sacs in the lung and causes the lung to become stiff and thick making it very hard for oxygen to reach the blood. Over time, the thickness and stiffness result in permanent scarring of lung tissue known as fibrosis and that makes it progressively more difficult to breathe. Pulmonary fibrosis can be caused by several factors but most times the causes of these changes are unknown and thus the terms “idiopathic”. Approximately more than 80,000 adults in the United States have idiopathic pulmonary fibrosis, and more than 30,000 new cases are diagnosed each year. Unfortunately, IPF there has no cure for IPF, and few treatment options are available to treat the symptoms.

What are the causes of Idiopathic Pulmonary Fibrosis?

  • The causes of IPF are not well understood but common risk factors include:
  • Family history
  • Cigarette smoking
  • Male sex
  • gastroesophageal reflux disease
  • Air pollution and work exposure to toxic chemicals
  • Age 60 and older

What are the symptoms of Idiopathic Pulmonary Fibrosis?

  • Progressive shortness of breath.
  • Progressive dry cough
  • unexplained weight loss
  • tiredness and fatigue
  • clubbing of the finger or toes
  • Rapid shallow breathing

How to diagnose Idiopathic Pulmonary Fibrosis?

Evaluation of IPF is usually made with the patient’s careful history and physical examination to determined patient lifestyle occupational exposure to toxic air pollutants. No laboratory tests are specific for a diagnosis of IPF and it is used mainly as a process of exclusion. Another diagnostic testing such as a chest x-ray, high-resolution CT scan, and lung biopsy may be required in the diagnostic work-up of IPF. A chest x-ray may increase in reticular markings which is also common with other interstitial lung diseases. High resolution computed tomography should be obtained in all patients presenting with IPF and common findings are basilar opacities associated with honeycombing and ground-glass opacities. Lung biopsy may show heterogeneous appearance with alternating areas of normal lung, fibrosis, fibroblast foci, and honeycomb change.

How to treat Idiopathic Pulmonary Fibrosis?

There is no cure for IPF and treatments are directed towards slowing the progression of the lung scarring. 

  • Nintedanib and Pirfenidone are two kinase inhibitors drugs approved to treat IPF around the world. These drugs slow the decline of lung functions and prevent acute exacerbations of IPF thereby prolonging survival
  • Antacids to treat gastroesophageal reflux to prevent acid reflux into the lungs which can worsen IPF.
  • Oxygen therapy and ventilator support: may be required to help with the work of breathing in the advanced stage
  • Surgery: A lung transplant may be done to replace 1 or both diseased lungs with healthy lungs.

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