Thinking About Idiopathic Pulmonary Fibrosis?

What is IPF?

Idiopathic pulmonary fibrosis is a specific form of chronic progressive fibrosing interstitial pneumonia of unknown etiology. IPF is a heterogeneous disease with variable clinical course.

When should we consider the possibility of IPF?

The risk of IPF should typically be considered in the case of the following combination of symptoms and findings:

• Progressive exertional dyspnea, loss of exercise tolerance, cough (patients may even be asymptomatic at the time of early diagnosis).
• Auscultatory finding of bilateral basal inspiratory crackles (crepitus, sometimes described as the sound of unfastening dry zip, the so-called velcro-rales phenomenon).
• Radiological finding of bilateral pulmonary fibrosis.
• Phenotypic manifestations (clubbing fingers with nails in the shape of a watch glass) occur in 75% of patients.

Risk factors of IPF

The risk factors of IPF are still being studied. Both non-modifiable and modifiable factors are likely involved in the pathogenesis of the disease. These risk factors may independently increase susceptibility to IPF or synergistically contribute to an increased risk of developing the disease. The following are considered risk factors:

• Older age: most cases of IPF are diagnosed in the 5th - 7th decade, about 2/3 of patients are over 60 years old (in younger patients, it may be familial IPF).
• Male gender: men represent up to 70% of IPF cases worldwide.
• Genetics.
• Smoking.
• Exposure to polluted air and other adverse environmental factors.
• Exposure to wood and metal dust, factors arising from stone, sand processing, and working with livestock.

Comorbidities also contribute to the risk of developing IPF, particularly the following:

• Gastroesophageal reflux: due to secondary and chronic microaspirations.
• Obstructive sleep apnea syndrome: significant fluctuations in pleural pressure may cause traction damage to alveoli.
• Diabetes mellitus: oxidative stress and excessive production of profibrotic cytokines are assumed.
• Herpetic infections: alveolar damage and fibrotic induction are assumed.

What needs to be ruled out?

If a patient with suspected IPF symptoms has no evidence of chronic obstructive pulmonary disease (COPD), asthma, or heart failure, they should be referred for examination to an IPF treatment center. One of the diagnostic criteria for IPF is the exclusion of other causes of interstitial lung processes (systemic connective tissue diseases, drug toxicity, household and occupational exposures). Collaboration with a rheumatologist is also advisable during the diagnostic process.

General principles of therapy

• The diagnosis and treatment of IPF in the Czech Republic are concentrated in centers for the treatment of interstitial lung processes.
• Pharmacotherapy involves the use of antifibrotic agents pirfenidone and nintedanib, which slow the decline in lung function.
• Essential components of therapy include treating tobacco dependence.
• Symptomatic reflux disease should be treated.
• Rehabilitation is conducted to strengthen respiratory muscles and improve exercise tolerance.

We are among the advanced countries in IPF treatment

The Czech Republic is considered very progressive in the approach to IPF treatment - up to 50% of domestic patients are treated with the most modern therapy. Since IPF is an incurable and debilitating disease, timely referral of the patient to a specialized center and the initiation of treatment that slows disease progression are crucial. The basis, however, is to consider the possibility of IPF, ideally already at the level of primary care.

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Sources:
1. Zaman T., Lee J. S. Risk factors for the development of idiopathic pulmonary fibrosis: a review. Curr Pulmonol Rep 2018; 7 (4): 118−125, doi: 10.1007/s13665-018-0210-7.
2. Šimůnková M. Czech advancements in the treatment of idiopathic pulmonary fibrosis. Remedia 2016; 26 (2): 193−194.
3. Vašáková M., Šterclová M. Idiopathic pulmonary fibrosis, recommended diagnostic, treatment, and monitoring procedure. Update 2016. Section of Interstitial Lung Processes ČPFS. Available at: www.pneumologie.cz/upload/1480186288.pdf