Idiopathic 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.

Related articles

 * Chronic lung diseases
 * Interstitial lung processes