Corticosteroid responsiveness in patients with acute exacerbation of interstitial lung disease admitted to the emergency department

Abstract

Interstitial lung disease (ILD), particularly idiopathic pulmonary fibrosis (IPF), has a poor prognosis. Corticosteroids are widely used in the treatment of acute exacerbation of ILD (AE-ILD). This study aimed to clarify the causes of AE-ILD, determine the efficacy of corticosteroids for treating AE-ILD, and detect differences in the mortality rate among subgroups of ILD. This was an observational retrospective single-center study. Patients with ILD who presented to the emergency department with acute respiratory symptoms from January 1, 2016, to December 31, 2018, were included. Patients with AE-ILD were classified into two groups depending on the prednisolone dose: low dose (0 to 1.0 mg/kg) or high dose (> 1.0 mg/kg). Mortality rates between patients with and without IPF were compared. This study included 182 patients with AE-ILD, including IPF (n = 117) and non-IPF (n = 65). Multivariate Cox regression analysis showed that corticosteroid dose (HR: 0.221, CI: 0.102–0.408, P < 0.001), initial P/F ratio (HR:0.995, CI:0.992–0.999, P = 0.006), and mechanical ventilation within 3 days of hospitalization (HR:4.205, CI:2.059–8.589, P < 0.001) were independent risk factors for mortality in patients with AE-ILD. This study showed that outcomes improve with higher doses of corticosteroids (> 1 mg/kg prednisolone) in patients with AE-non-IPF-ILD. However, this was not the case in patients with AE-IPF.

Management of Connective Tissue Disease–related Interstitial Lung Disease

“…The dose and duration need to be individualized. Caution is warranted as the use of corticosteroids in IPF is associated with increased mortality [ 52 ].…”

Interstitial lung disease on the acute take for the non-respiratory physician

“…AEs in IPF have a particularly poor outcome with an in-hospital mortality of greater than 50% and high post-event morbidity. 8,13 It is uncertain what triggers them. There may be some unidentified intrinsic factor that causes more rapid disease progression or they may simply represent a response to external factors (such as infection, aspiration, pulmonary embolism or post-anaesthesia for surgery).…”

“…One recent study, by Jang et al, has provided data from a retrospective review of AE-ILDs suggesting that low-dose steroids (<1 mg/kg) are preferable in IPF-AE but that for non-IPF-AE higher-dose steroids may be more effective. 13 Mounting evidence is pushing clinicians away from an aggressive immunosuppressive approach in AE-IPF. The authors favour a lowdose steroid approach of 30-40 mg/day for 10-14 days until further research data are available.…”

“…PJP infection is e589 CME: Respiratory medicine more common in patients with ILD on IS drugs (16%) than those with ILD who are not on IS drugs (<3%). 13 In summary, treatment of AE-ILD remains difficult due to a lack of high-quality evidence. Clinicians should consider broad spectrum antibiotics or treatment for other specific infections identified in all exacerbating patients, at least initially, particularly in those who are immunosuppressed.…”

Krebs von den Lungen‐6, a promising marker in evaluating the severity of interstitial lung disease in Egyptian rheumatoid arthritis patients

“…10 ILD is characterized by parenchymal inflammation and/or fibrosis of the interstitium, alveoli or terminal bronchioles. 11 ILD is the most common form of RA associated pulmonary diseases and it is characterized by diffuse pulmonary parenchymal inflammation and interstitial fibrosis. ILD occurrence may precede articular manifestations.…”