Beyond Classification: An Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Case

TL;DR

This paper presents a case of a patient with features overlapping two types of vasculitis, highlighting the need for individualized treatment approaches.

Contribution

The paper introduces a case that challenges current classification systems by showing an overlap between GPA and EGPA.

Findings

The patient exhibited features of both GPA and EGPA, including eosinophilia and PR3 c-ANCA positivity.

Treatment with glucocorticoids and cyclophosphamide led to clinical improvement and remission.

The case suggests a GPA-EGPA overlap phenotype exists and requires personalized treatment strategies.

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are usually classified as distinct entities, such as granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In everyday practice, however, some patients display overlapping features of both conditions, making classification and treatment decisions more challenging.

We report a case of a 51-year-old man with late-onset asthma who presented with constitutional symptoms, purulent rhinosinusitis, hemoptysis, and arthralgia. Imaging demonstrated cavitary pulmonary nodules and nasal polyposis. Laboratory testing showed marked eosinophilia and positivity for proteinase 3 (PR3) c-ANCA. Nasal biopsy revealed necrotizing granulomatous inflammation rich in eosinophils. The patient received induction therapy with high-dose glucocorticoids and cyclophosphamide, followed by rituximab for maintenance, with clinical improvement and sustained remission. This case highlights the limitations of current classification frameworks and is compatible with a GPA-EGPA overlap phenotype (or spectrum). It underscores the value of an individualized approach guided by the predominant organ-threatening manifestations and the associated biological profile.

Figures