IVIG May Improve Survival in Patients with Acute Exacerbation of Fibrotic Idiopathic Interstitial Pneumonia

Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a frequently fatal condition, particularly in patients with a lower percent-predicted forced vital capacity within 12 months before AE onset and/or a lower PaO2/FiO2 ratio at AE onset. Currently, there are no established treatments beyond a weak recommendation for treatment with high-dose corticosteroids. Investigators at Okayama University Hospital and KKR Takamatsu Hospital in Japan hypothesized that intravenous immune globulin (IVIG), which has both anti-inflammatory and anti-infective effects, may have a positive effect on AE of fibrotic idiopathic interstitial pneumonias (IIPs), including IPF.

They retrospectively analyzed 52 consecutive patients diagnosed with AE of fibrotic IIPs and treated with pulse corticosteroid (methylprednisolone 500 to 1000 mg/day for three days) between April 2018 and May 2021 at their two institutions. Thirteen of the 52 patients received IVIG (5 g/day for three to five days) concurrently with pulse corticosteroid therapy. The remaining 39 patients were assigned to the control group.

The survival rate on day 90 was significantly higher in the IVIG group than in the control group (76.9 percent versus 38.5 percent, p = 0.02). IVIG administration (odds ratio [OR], 0.11; 95 percent confidence interval [CI], 0.02-0.69; p = 0.02) and C-reactive protein (OR, 1.19; 95 percent CI, 1.06-1.22, p < 0.01) were independently associated with 90-day mortality.

Encouraged by this evidence that administration of IVIG may improve 90-day survival of patients with AE of fibrotic IIPs, in May 2022, the study investigators initiated a prospective, randomized clinical trial in hopes of confirming this treatment effect.