Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease

ABSTRACT

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.

Areas covered: We reviewed EGPA clinical presentation, focusing on clinical manifestations at three different phases of the disease: 1/before the development of overt vasculitis, 2/at vasculitis diagnosis and 3/during the long-term follow-up. An update on current classification criteria and recent therapeutic advances has been provided as well.

Expert opinion: Asthma, chronic rhinosinusitis and blood eosinophilia could anticipate the overt vasculitis for years. An atopic background may be present in a subset of patients (25-30%), while ANCA presence varies between 10 and 40%. Systemic vasculitis rapidly occurs and clinical features demonstrating vasculitis processes (neuropathy, purpura, scleritis, alveolar hemorrhage and glomerulonephritis) develop along with systemic symptoms (50%). After vasculitis resolution, asthma remains severe in up to 50% of patients and incidence of isolated-asthma and rhinosinus exacerbations remains constantly high. Different sets of classification criteria have been published so far, and DCVAS diagnostic criteria will be presented soon. Interleukin-5 blockers seem to be promising to control the disease and to spare corticosteroids.

KEYWORDS:

• Severe/uncontrolled asthma
• eosinophilic asthma
• eosinophilic granulomatosis with polyangiitis
• EGPA
• Churg-Strauss
• ANCA-associated vasculitis
• long-term outcomes
• vasculitis
• mepolizumab
• Interleukin-5

Article highlights

• In EGPA patients, before overt vasculitis, severe asthma, chronic rhinosinusitis ± nasal polyposis and blood eosinophilia dominate the clinical presentation, while an atopic background may be present 25-30% of patients, and circulating ANCA (usually directed against myeloperoxidase) in 10-40%.

• At vasculitis onset, clinical features demonstrating a vasculitis process (peripheral nervous system involvement, purpura, scleritis, alveolar haemorrhage and extra-capillary glomerulonephritis) develop along with systemic symptoms. Vasculitis manifestations seem to be more frequent in ANCA-positive patients.

• During long-term follow-up, asthma remains severe and/or steroid dependent in up to 50% of patients. Despite treatment and corticosteroid use, isolated asthma and rhinosinusitis exacerbation rates remain high long after the resolution of vasculitis.

• To date, different sets of classification criteria have been published, and DCVAS diagnostic criteria will be released soon.

• Anti-interleukin 5-targetted biologics are promising to control the disease and to spare corticosteroids, yet their ability to curb vasculitis manifestations remains unclear.

Acknowledgments

We thank Francesca Dallago for her contribution in figure production.

Declaration of interest

M Groh has received consulting fees from GlaxoSmithKline and AztraZeneca. 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.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.