Idiopathic pulmonary fibrosis

HRCT pulmonary fibrosis

It is a diffuse, primarily fibrotic lung process.

Pathogenesis

This is probably a uniform pathological response of lung tissue to both infectious and non-infectious agents. These cause damage to the lining of the alveoli and thus result in progressive and uncontrollable scarring. The inflammatory reaction as such can occur only secondarily.

Epidemiology

• Patients are most often between the ages of 40 and 70.
• The incidence in women is 7.4 / 100,000 and in men 10.7 / 100,000.
• It occurs sporadically, is equally widespread in all localities, familial cases are rare.
• The disease is practically incurable, and even with adequate treatment, survival usually does not exceed 3-5 years.

Clinical picture

• Onset – rolonged unproductive cough in time with worsening exertiona dyspnea, fatigue, weight loss, tachypnoe;
  • on the bases of lungs late inspiratory crepitus similar to Velcro opening;
  • eventually chronic hypoxia with cyanosis develops.
• In 2/3 of the patients there are club-shaped fingers with nails in the shape of a watch glass.
• Image of COPD without obstructive defect, in the later phase restrictive lung damage - reduction of FVC.
• Despite the typically protracted progressively deteriorating course, acute exacerbations may occur in some patients:
  • sudden clinical deterioration;
  • decreased lung function;
  • radiological image of the so-called milk glass (indicating alveolitis).

Diagnostics

Here, HRCTs are crucial, and a typical clinical finding does not require a biopsy if systemic connective tissue diseases and an exogenous cause are excluded.

• HRCT image of the lungs: pulmonary fibrosis with an image of the honeycomb lung in the bases of the lungs and minimal areas of active changes.
• Histology from a lung biopsy.
• In patients unable to undergo surgical biopsy, X-ray and bronchoscopy must be sufficient.
• X-ray: increased lung drawing to reticulation - honeycomb lung.
• Functional examination: restrictive ventilation disorder, pulmonary compliance disorder.

Therapy

• Anti-inflammatory and immunosuppressive drugs are ineffective because the main pathological mechanism here is pathological fibroproduction, so they are not used in treatment today.
• Pirfenidon – inhibits fibrosis, indicated in patients with FVC 50-80%. Dosage 3x3cps - a total of 2403 mg.
• Nintedanib – a tyrosine kinase inhibitor on VEGFR, FGFR, and PDGFR
• Early alveolar lesions: N-acetylcystein 3 times 600 mg (antioxidant effect).
• Acute exacerbations: high doses of corticoids, anticoagulant therapy, and antibiotics. PPI (proton pump blockers) are given to prevent exacerbations.
• Advanced diseases with hypoxemia: long-term home oxygen therapy and consideration of lung transplantation.
• Corticosteroids in long-term therapy are ineffective, pbecause fibrotization is not induced by an inflammatory response.

Summary video

Idiopathic pulmonary fibrosis (video in english)

Links

Related articles

• Chronic lung diseases
• Interstitial lung processes

Sources

• BENEŠ, Jiří. Studijní materiály [online]. [cit. 2010]. <http://jirben.wz.cz>.

• ČEŠKA, Richard, et al. Interna. 1. vydání. Praha : Triton, 2010. 855 s. ISBN 978-80-7387-423-0.

• VAŠÁKOVÁ, Martina. Léčba idiopatické plicní fibrózy. Remedia [online]. 2012, roč. 2012, vol. 2, s. 398-402, dostupné také z <http://www.remedia.cz/Clanky/Farmakoterapie/Lecba-idiopaticke-plicni-fibrozy/6-L-1oN.magarticle.aspx>. ISSN 0862-8947.