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Disease Characteristics

Pulmonary fibrosis (PF) is a type of chronic lung disease that sees an irreversible decline in a patient’s lung function. The condition is characterised by scarring (fibrosis) of the lung tissue. This scarring gets progressively worse over time, and in advanced cases can see the patient being unable to take in enough oxygen through the lungs.

It is defined as an ‘interstitial’ lung disease, affecting only the lung tissue around the air sacs of the lungs (the interstitium). The blood vessels and airways of the lungs remain largely unaffected. In this way IPF is similar to many other types of interstitial lung disease, but can be treated very differently. Accurate diagnosis is crucial to minimising the effects of PF.

There are two main types of pulmonary fibrosis:

  • Idiopathic Pulmonary Fibrosis (IPF)
  • Familial Pulmonary Fibrosis (FPF) 

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Risk factors of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is characterised by its causes being largely unknown (‘idiopathic’ literally meaning ‘of unknown cause’). There are, however, a range of common environmental and exposure risk factors that have been linked to increased instances of the disease, including:

  • Cigarette smoking: Active or former smokers have a 1.6 times greater risk of developing IPF than non-smokers.1
  • Age and sex: IPF sufferers are more often male, and almost all are over the age of 50.2,3
  • Acid reflux and heartburn: Many patients experience symptoms of gastroesophageal reflux disease (GERD) and heartburn.4
  • Environmental exposures: Exposure to dusts, including wood, metal, biological, silica, stone, and hay, have been shown to increase the incidence of IPF.5,6,7,8

It is a rare disease with an incidence estimated at 3.81 per 100,000 inhabitants in Europe.9

Symptoms of Idiopathic pulmonary fibrosis

There are many symptoms that could lead to a diagnosis of idiopathic pulmonary fibrosis, including:

  • Breathlessness
  • A persistent dry cough
  • A Velcro-like sound in the lungs when inhaling
  • ‘Clubbing’ of the fingers and toes (swelling at the extremities)
  • Abnormal results in pulmonary function tests
  • Chest tightness and pain
  • An unexplainable loss of appetite (and subsequently, weight)
  • Lethargy

Many of these symptoms aren’t unique to IPF and may simply point to hypoxemia (oxygen deficient blood). As an idiopathic disease, diagnosis necessitates first ruling out other, better understood causes.

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FAMILIAL PULMONARY FIBROSIS

Familial pulmonary fibrosis is defined by the presence of at least two cases of pulmonary fibrosis in the same family10,11. Up to one in five sufferers12 of pulmonary fibrosis have a family history of the disease, meaning that the cause shifts from idiopathic (unknown) to familial. However treatment for both diseases remains largely the same.

TREATMENT OF PULMONARY FIBROSIS

GENERAL

The most common symptoms of pulmonary fibrosis – a dry cough, shortness of breath and acid reflux – can be eased through a variety of medications. Respiratory failure is the primary concern for IPF patients, but the disease can also lead to conditions such as heart failure, pulmonary hypertension, pneumonia and lung cancer, signs of which must be acted on quickly. Despite medical advances, PF still sees high in-hospital mortality.13 The vagaries of the disease can present a number of challenges to clinicians in a real-life setting.14

CRITICAL CARE

Since mechanical ventilation is associated with high mortality and poor outcoes reported in patients with pulmonary fibrosis15,16, it is not always indicated for patients with acute exacerbation, and it is highly recommended to provide the information regarding the limitations and poor outcomes of mechanical ventilation to patients and their families.17,18

With a mean survival time of 3-5 years post-diagnosis,19 lung transplatation represents the most promising option, but many critical care institution are simply not equipped for such a procedure, and indeed, the patient may not be a suitable candidate.

Dyspnoea, and the resultant hypoxia, is common in patients with PF. Oxygen supplementation is currently recommended by guidelines.18 Invasive mechanical ventilation should be considered only if used as bridge for preplanned lung transplantation.20,21

Noninvasive ventilation is the preferred option for acute exacerbation and acute respiratory failure in IPF patients.22,23

Discover below which respiratory and monitoring products from Medtronic can help in the treatment of patients who have been diagnosed with pulmonary fibrosis.

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  • 2. Kim D, Collard H, King T. Classification and Natural History of the Idiopathic Interstitial Pneumonias. Proceedings of the American Thoracic Society. 2006; 3(4): 285–292.
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