Idiopathic pulmonary fibrosis patients experiencing acute exacerbations (IPF-AE) who have a history of immunosuppression and high-dose steroid use are likely to have measurably worse survival outcomes than IPF-AE patients not under such treatment, a new study reported. The research article, “Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach,” was published in the journal BMC Pulmonary Medicine.
IPF, a chronic interstitial lung disease affecting the tissue that supports the alveoli in the lungs, is characterized by the inflammation or accumulation of scar tissue (fibrosis), leading to progressive loss of elasticity, failure of proper lung activity, and insufficient oxygenation of the blood and organs. IPF’s clinical course and progression is highly variable among patients, and the trajectory and rate of decline are difficult to predict at diagnosis. IPF-AE is the most serious event during disease and can cause acute respiratory distress. Current therapies include the prescription of steroids and other immunosuppressants, although no significant literature exists as to their effectiveness.
For this reason, the research team, led by Effrosyni Manali with the Athens Medical School, National and Kapodistrian University of Athens, Greece, hypothesized that previous immunosuppression and the administration of high-dose steroids might adversely affect IPF-AE outcome. The medical team also reported in its study that it has “abandoned in our everyday clinical practice” that therapy approach.
Researchers studied 85 patients hospitalized between 2007 and 2013 for IPF deterioration and applied a specific protocol, where immunosuppression therapy was stopped and best supportive care was offered, including broad-spectrum antimicrobials, medical evaluation, and assessment of possible reversible causes of health deterioration. Among this patient population, 24 patients (28 percent) fulfilled the criteria for IPF-AE.
IPF-AE analysis was conducted as unique events and unique patients. The researchers determined that, as unique events, patients had a 50 percent survival rate, and among those who survived, 25 percent had previously undergone immunosuppression and 75 percent had not received immunosuppression therapy. Analyzed as unique patients, data showed that 35.3 percent survived, with 50 percent never having been immunosuppressed and 27.3 percent having receiving immunosuppression.
After discharge, the IPF-AE patients evaluated in the study had an 83 percent one-year survival. Survival rates were again greater among non-treated patients compared to immunosuppressed patients, showing that immunosuppression adversely influenced survival in a significant way.
Researchers concluded that, “by applying the critically ill patient protocol on the management of IPF patients developing acute exacerbation we have shown that half of our patients survived. Furthermore, we found that the history of immunosuppression before IPF-AE adversely influences survival. Avoiding steroids in IPF patients may favor the natural history of the disease even at the moment of its most devastating event.”