Researchers Discuss Idiopathic Pulmonary Fibrosis Comorbidities

A team of researchers from the Department of Clinical and Experimental Medicine at the University of Catania, Italy recently reviewed the co-occurrence of additional diseases in Idiopathic Pulmonary Fibrosis and their current therapeutic strategies. The study, entitled “IPF, comorbidities and management implications,“ was published in the Sarcoidosis Vasculitis and Diffuse Lung Disease journal.

Idiopathic Pulmonary Fibrosis (IPF) is a progressive life-threatening lung disease characterized by the thickening and scarring of pulmonary alveoli, affecting oxygen transfer to the bloodstream. IPF is not curable and available treatments are only used to aid patients in breathing. IPF is associated with a wide variety of comorbidities of the respiratory and non-respiratory system. In this recent study, the team focused on three common diseases linked to IPF diseases — cardiovascular disorders, lung cancer and emphysema.

Cardiovascular disorders are the most common comorbidity in IPF, increasing disease mortality. Researchers are still not certain whether it is IPF that leads to cardiovascular deterioration or if the opposite is true, since evidence supports both hypotheses. Regardless of the cause, the onset of a prothrombotic state is central in IPF and cardiovascular disorder concomitance. The use of anticoagulants to reduce the thrombosis risk in IPF patients with cardiovascular disorders is nonviable. Studies show that treatment is ineffective and further increases patient mortality.

Lung cancer prevalence in IPF patients ranges from 5% to 20% with its cumulative incidence increasing over time with poor diagnosis. IPF and lung cancer share many risk factors — advanced age, male gender and smoking — as well as genetic and pathogenic pathways. It is extremely difficult to decide whether to treat and how lung cancer in IPF patients, since cancer treatment is associated with a significant toxicity and acute IPF exacerbations. Patient management in these cases should include the annual monitoring of cancer growth, reduction of risk factors (e.g., smoking) and the regular evaluation of lung function and performance. In specific patients who have a less advanced cancer stage, surgery or radiotherapy might be useful.

Co-occurrence of IPF and emphysema represents a clinical syndrome termed combined pulmonary fibrosis and emphysema (CPFE), which is less frequent than cardiovascular and lung cancer comorbidities. Causes of CPFE are heterogeneous with male gender and smoking predominance, with studies suggesting also a genetic predisposition. There is no direct cause implicating emphysema in IPF patient mortality. Nonetheless, this syndrome is associated with a variety of comorbidities and complications, also increasing the risk of lung cancer development. Currently, there are no specific treatments for CPFE, with clinicians recommending smoke cessation and the use of bronchodilators.

As final recommendations, authors propose that patients with IPF should be carefully evaluated for comorbidities, since they worsen disease prognosis and mortality. The authors also argue for the development of new trials to assess novel therapies against IPF comorbidities.

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