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New treatments in idiopathic pulmonary fibrosis

Dr Irina Strambu

Abstract: 

Idiopathic pulmonary fibrosis is a chronic fibrotic pulmonary disease of unknown origin, with an unfavourable prognosis, leading to death by respiratory failure in most patients within 3-5 years ofdiagnosis. Several drugs were studied for the treatment of this disease, and most of them were not able to stop the relentless evolution of the disease (warfarin, corticosteroids in combination with azathioprine, N-acetylcysteine, ambrisentan, bosentan, sildenafil, antiacids). Two novel drugs, pirfenidone and nintedanib, proved effective in reducing lung function decline, improving the patient’s quality of life, and increasing the patient’s probability of survival. These drugs were approved by international health authorities for use in the treatment of IPF patients. The paper refers also to the status of IPF patients in Romania, where epidemiological data are not known, and where the disease is most likely severely underdiagnosed. Patients are typically diagnosed late, and are therefore in advanced stages of the disease. A proactive attitude, in favour of identification and early diagnosis of IPF patients is highly needed in order to offer to these patients the opportunity of treatment, improved survival, and a better quality of life.
 

Keywords: 
idiopathic pulmonary fibrosis, pirfenidone, nintedanib  

Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic pulmonary disease of unknown cause affecting adults. IPF is one of the most aggressive interstitial diseases, having a progressive, relentless, and relatively rapid evolution towards an invalidating lung fibrosis that induces respiratory failure and premature death, typically within 4 years after diagnosis. From this per-
spective, IPF is comparable with most aggressive cancers.

IPF is included in the group of idiopathic interstitial pneumonias, according to the classification of the International Committee for IPF (1) .

 

References: 
  1. Raghu G, Collard HR, Egan JJ et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Resp Crit Care Med. 2011;183(6):788-824.
  2. Raghu G, Weycker D, Edelsberg J et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Resp Crit Care Med. 2006; 174(7):810-816).
  3. Ley B, Collard HR. Epidemiology of idiopathic pulmonary fibrosis. Clinical Epidemiology. 2013:5 483-492.
  4. www.regis.ro

 

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