Steroid alternatives for managing eosinophilic lung diseases

ABSTRACT

Introduction

Chronic pulmonary eosinophilia is a rare condition, usually highly responsive to systemic glucocorticoids, yet relapses are frequent and require long-term treatment associated with significant morbidity.

Areas covered

We review the main conditions causing chronic pulmonary eosinophilia and glucocorticoid-sparing agents in this setting.

Expert opinion

An individually tailored etiologic assessment is mandatory in all patients presenting with chronic pulmonary eosinophilia. Reducing the cumulative exposure to glucocorticoids by treating flares with short courses of systemic glucocorticoids is both safe and feasible in idiopathic chronic eosinophilic pneumonia. In frequently relapsing patients and in those requiring high doses of glucocorticoids, alternatives to glucocorticoids should be considered. Mepolizumab has been approved in several countries for the treatment of both hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis. Given their ability to induce rapid and sustained depletion in blood and tissue eosinophils (e.g. benralizumab and lirentelimab) and/or to curb Type 2-mediated inflammation (e.g. dupilumab), other biologics hold promise in these settings. Likewise, anti-IgE targeted therapies (i.e omalizumab) should be considered in the glucocorticoid-sparing therapeutic armamentarium of allergic or hypersensitivity-related diseases, including allergic bronchopulmonary aspergillosis. Other drugs (fevipiprant, tezepelumab) are at an early phase of development. Cost-effectiveness studies are lacking. Whether glucocorticoid-free treatment regimens are achievable in these conditions is unknown.

KEYWORDS:

• Allergic bronchopulmonary aspergillosis
• biotherapies
• eosinophilic granulomatosis with polyangiitis
• hypereosinophilic syndrome
• idiopathic chronic eosinophilic pneumonia
• interleukin-5
• systemic glucocorticoids

Article highlights

• Chronic pulmonary eosinophilia encompasses a wide spectrum of diseases (including idiopathic chronic eosinophilic pneumonia, eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, allergic bronchopulmonary aspergillosis, and IgG4-related diseases) whose clinical presentation can overlap.

• As these conditions require specific treatments, a stepwise diagnostic and etiologic workup is mandatory in all patients with chronic pulmonary eosinophilia.

• On-demand short courses of systemic glucocorticoids (without long-term maintenance therapy) are both safe and feasible for the treatment of idiopathic chronic eosinophilic pneumonia.

• Mepolizumab was recently approved by the FDA for the treatment of both idiopathic hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis.

• Other biologics (e.g. benralizumab, dupilumab, tezepelumab) targeting type 2-mediated cytokines are promising treatments for conditions associated with chronic pulmonary eosinophilia.

This box summarizes key points contained in the article.

Declaration of interest

M Groh has received consulting fees from GlaxoSmithKline and AstraZeneca. M Nasser has received lecture fees from AstraZeneca. JE Kahn has received consulting fees from GlaxoSmithKline and AstraZeneca. V Cottin has received lecture and consulting fees from AstraZeneca. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

The authors have no funding to report.