Idiopathic pulmonary fibrosis

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HRCT pulmonary fibrosis

It is a diffuse, primarily fibrotic lung process.

Pathogenesis[edit | edit source]

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[edit | edit source]

  • 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[edit | edit source]

  • 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[1];
    • 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[edit | edit source]

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[edit | edit source]

  • 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. [2][3][4]

Summary video[edit | edit source]

Idiopathic pulmonary fibrosis (video in english)

Links[edit | edit source]

Related articles[edit | edit source]

Reference[edit | edit source]

  1. KOLEKTIV, Marek. Farmakoterapie vnitřních nemocí - 4. zcela přepracované a doplněné vydání. - vydání. Grada Publishing a.s., 2010. 777 s. s. 170. ISBN 9788024726397.
  2. BENEŠ, Jiří. Studijní materiály [online]. [cit. 2010]. <>.
  3. ČEŠKA, Richard, et al. Interna. 1. vydání. Praha : Triton, 2010. 855 s. ISBN 978-80-7387-423-0.
  4. VAŠÁKOVÁ, Martina. Léčba idiopatické plicní fibrózy. Remedia [online]. 2012, roč. 2012, vol. 2, s. 398-402, dostupné také z <>. ISSN 0862-8947. 

Sources[edit | edit source]

  • BENEŠ, Jiří. Studijní materiály [online]. [cit. 2010]. <>.
  • 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 <>. ISSN 0862-8947.