In a study entitled “Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis,” the authors highlight the difficulty associated with the diagnosis of acute exacerbation of idiopathic pulmonary fibrosis. The study was published in Respiratory Research journal.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by scarring of lung tissue that leads to shortness of breath and ultimately death after 2 to 3 years after diagnosis, as a result of progressive respiratory failure. During IPF’s clinical progression, periods of acute deterioration in respiratory function, termed acute exacerbation of IPF (AE-IPF) can occur, and are associated with an extremely poor prognosis.

Patients with IPF exhibit histological focal areas known as usual interstitial pneumonia (UIP) with fibroblast-myofibroblast aggregates termed fibroblastic foci. In the case of AE-IPF, these are usually associated with diffuse alveolar damage (DAD). However, several studies identified organizing pneumonia (OP) without the occurrence of diffuse alveolar damage (DAD) or fibroblastic foci. In this study, the authors performed a retrospective review of postmortem pathological findings of patients with AE-IPF, to identify the characteristics of “acute exacerbation” in AE-IPF patients.

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For that, the authors reviewed autopsies performed between 1999 and 2013 from 52 consecutive patients with AE-IPF, from which 45 were submitted to high-dose corticosteroid therapy after diagnosis. The authors found that AE-IPF patients exhibit several pathological features besides diffuse alveolar damage (DAD). They also found that AE-IPF patients are frequently diagnosed with infectious diseases, including fungus, cytomegalovirus, and bacteria. Additionally, they found that fibrotic lesions presented the usual interstitial pneumonia pattern in all the patients, but other complications were also observed, including diffuse alveolar damage (DAD), pulmonary hemorrhage, pulmonary thromboembolism, and even patients with lung carcinoma. The authors observed patients with pneumothorax prior to death and some patients developed insulin-dependent diabetes after initiating treatment with corticosteroids.

The authors suggest that, in light of their findings, AE-IPF is characterized by multiple pathological conditions besides diffuse alveolar damage (DAD), which makes the diagnosis challenging. Due to these complications during the clinical course of the disease, AE-IPF patients have a higher risk of death. Thus, these patients require constant monitoring of the condition as well as the applied therapeutics.