Immunosuppressants Harm IPF Patients with Acute Exacerbations, Study Reports

In a new study, clinicians propose a new treatment protocol without immunosuppressants or high-dose steroids to manage and treat acute exacerbations in patients with idiopathic pulmonary fibrosis (IPF). The study, titled “Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach,” was published in the journal BMC Pulmonary Medicine.

IPF is a severe and irreversibly progressive lung disease marked by acute exacerbations or flares. Although IPF’s etiology is unknown, in the last few decades IPF patients have received steroids and other immunosuppressants (especially azathioprine) as a treatment; however, there are no studies proving the effectiveness of these drugs in IPF flares, and some studies actually suggest they may cause harm.

A group of clinicians investigated whether a history of previous immunosuppression together with the administration of high-dose steroids adversely affected the outcome of IPF acute exacerbations. To this end, the team studied all patients with a diagnosis of pulmonary fibrosis admitted to Attikon University Hospital in Athens, Greece, from January 2007 to June 2013. The team identified the cases of IPF and, within these, selected patients with IPF acute exacerbations (according to the IPF Clinical Research Networks Investigators consensus), recognized by an IPF diagnosis, unexplained worsening or development of dyspnea (shortness of breath) within 30 days, new lung infiltrates, and no identifiable or treatable cause of lung injury.

The patients were immediately submitted to a “new” protocol consisting of an immediate termination of immunosuppression therapy (in the cases where it was being administered), improved supportive care (including continuous monitoring of the patient, oxygen supplementation, painkillers, and anti-febrile medications), administration of broad spectrum antimicrobials (drugs that act against a wide range of disease-causing bacteria), and a close evaluation to detect reversible causes of deterioration. All patients continued to be followed after discharge, and immunosuppression therapy was not administered even post-discharge.

In total, out of the 85 IPF admissions, 24 fulfilled the IPF acute exacerbations criteria (28%). While only 27.3% of the patients previously treated by immunosuppression survived, the survival rate increased to 50% in the group not receiving immunosuppression. The clinicians concluded that “immunosuppression significantly and adversely” influences survival, with patients who were never treated with immunosuppressants responding better to the new protocol and achieving higher survival rates. Moreover, after discharge, IPF acute exacerbations patients in the never-treated group had a one-year survival rate of 83%.

The clinicians highlighted that with their new protocol half of the patients with IPF acute exacerbations survived, and that a history of immunosuppression impairs survival. Moreover, withholding high-dose steroid and substituting it with broad-spectrum antimicrobials seems to benefit survival in IPF acute exacerbation patients.