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Health Protocols

Idiopathic Pulmonary Fibrosis


Early diagnosis and initiation of treatment to slow the loss of lung function offers the best prognosis in IPF (Scelfo 2017). Most IPF patients are older men and their histories often include risk factors such as cigarette smoking, certain occupational exposures, or gastroesophageal reflux disease (Salvatore 2018). They typically describe experiencing shortness of breath that has gradually worsened over years, sometimes with periods of acute exacerbation. Other common symptoms include (Tzilas 2017; Scelfo 2017; Cottin 2015; Atkins 2016; Vainshelboim 2016):

  • Dry cough
  • Difficult breathing
  • Painful breathing
  • Fatigue
  • Weight loss
  • Weakness of respiratory and other muscles
  • Anxiety and depression

Physical exam will reveal crackles, a type of popping or "crackling" sound emanating from the lungs that can be heard with a stethoscope (Vyshedskiy 2012; Sarkar 2015). Clubbing, a deformity of the fingers and toes frequently associated with lung diseases, may be seen in as many as 50% of IPF cases (Tzilas 2017; Sarkar 2012; Magazine 2012). Pulmonary function tests usually, but not invariably, demonstrate restricted lung expansion (Salvatore 2018; Tzilas 2017). If the history and exam findings suggest fibrotic lung disease, other possible causes, such as medication toxicity, sclerotic diseases, sarcoidosis, and exposure to radiation, asbestos, silica, mold, or other known fibrosis triggers, need to be explored as part of achieving an accurate diagnosis and identifying an appropriate course of treatment (Tzilas 2017; Sharif 2017).

High-resolution computed tomography (CT) scan is the next diagnostic step in patients with suspected IPF. A fibrotic pattern known as usual interstitial pneumonia, with a characteristic honeycomb appearance, is an indication of interstitial scarring and, in the absence of another known cause, confirms the diagnosis of IPF without the need for a surgical lung biopsy (Salvatore 2018). If findings from high-resolution CT scan are inconclusive, a lung biopsy can be performed to make a certain diagnosis; however, because of its highly invasive nature, lung biopsy is generally reserved for those in whom a clear diagnosis is likely to affect treatment decisions and those deemed resilient enough to endure such a procedure (Tzilas 2017; Sharif 2017).