Current Therapy Options for Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a chronic progressive disease that exclusively affects adults, predominantly of middle and older age. Until recently, the disease was practically untreatable. However, this has changed with the discovery of antifibrotic drugs, which have significantly altered the prognosis for patients. Early diagnosis and initiation of therapy at a specialized center, of which there are several in the Czech Republic, are essential for success.
Clinical Presentation
Key clinical symptoms include progressive exertional, and later, resting dyspnea, easy fatigue, cough, and in later stages, cyanosis due to hypoxemia. Clubbed fingers with nails resembling watch glass are present in 75% of patients, and crepitus over lung bases is heard on auscultation. Spirometry typically shows findings of restrictive lung disorder with significant lung compliance impairment and reduction of transfer factor TLCO.
The disease affects only lung tissue, with no systemic symptoms present. The disease is characterized by a gradual progression of symptoms; however, some patients experience acute exacerbations characterized by worsening lung function and radiological findings of ground-glass opacities.
Diagnosis
A multidisciplinary approach involving a pulmonologist, an experienced radiologist, and, if necessary, a pulmonary pathologist is required to diagnose the disease. Idiopathic pulmonary fibrosis is diagnosed based on clinical and imaging examinations, excluding other etiologies of symptoms.
The primary diagnostic method is high-resolution computed tomography (HRCT) of the lungs. The IPFchecker online application, which includes a risk calculator for idiopathic pulmonary fibrosis and the option to consult findings with an experienced radiologist, can be used for diagnosis.
Other diagnostic tools include spirometry, bronchoalveolar lavage for differential diagnosis, and pulmonary biopsy in case of ambiguities.
Current Therapy Options
The discovery of antifibrotic drugs was a significant breakthrough in therapy, but the prognosis of the disease remains severe and comparable to that of lung cancer. Patients typically die from respiratory failure 2–5 years after diagnosis.
Early diagnosis coupled with the implementation of effective antifibrotic therapy increases survival. The first approved drug in this group was pirfenidone, which was designated as a breakthrough therapy for idiopathic pulmonary fibrosis by the U.S. Food and Drug Administration (FDA). The drug is covered for patients with forced vital capacity (FVC) of 50-90% and TLCO >30%. Another drug in the antifibrotic group is the tyrosine kinase inhibitor nintedanib. Antifibrotic therapy is administered in centers for the diagnosis and treatment of interstitial lung diseases under the auspices of the Czech Pneumological and Phthiseological Society ČLS JEP.
Supportive therapy methods include the administration of N-acetylcysteine (antioxidant effect), proton pump inhibitors (prevention of acute exacerbations), opioids (alleviation of dyspnea in terminal stages), home oxygen therapy (for advanced disease with hypoxemia), and rehabilitation. Lung transplantation is also suitable for strictly selected patients.
Conclusion
Idiopathic pulmonary fibrosis is a progressive lung tissue disease with a severe prognosis. Early diagnosis combined with the initiation of antifibrotic therapy is critical for increasing the chance of longer survival. Every patient suspected of idiopathic pulmonary fibrosis should be referred to a center for the diagnosis and treatment of interstitial lung diseases.
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Source: Vašáková M. Treatment of idiopathic pulmonary fibrosis in the Czech Republic in 2015. Internal Medicine 2015; 17 (3): 128–132.
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