Asthma-free eosinophilic granulomatosis with polyangiitis presenting with Guillain-Barré syndrome-like symptoms: a...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis classically characterized by asthma, eosinophilia, and necrotizing vasculitis. Although the vast majority of EGPA patients present with concomitant asthma, this feature should not be considered an absolute prerequisite for diagnosis.

A 50-years-old male presented with a 2-weeks history of progressive numbness and weakness in all four extremities, leading to an initial admission under the provisional diagnosis of Guillain-Barré syndrome (GBS). Although the subacute disease course was confounding, physical examination at our institution identified two pivotal clues: marked asymmetric involvement and severe neuropathic pain. These findings strongly suggested the possibility of vasculitic neuropathy. The patient lacked classical pulmonary manifestations, such as asthma or nasal polyposis, and pulmonary function tests were within normal limits. Nevertheless, his asymmetric mononeuritis multiplex, cutaneous rash, and marked eosinophilia (3.33 × 10^∧9/L, 18.9%) constituted critical diagnostic evidence. A subsequent skin biopsy revealed characteristic vasculitic changes. By applying the 2022 ACR/EULAR classification criteria, the patient's cumulative score firmly supported the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). The patient achieved symptomatic remission following a 3-months therapeutic regimen comprising systemic glucocorticoids and mycophenolate mofetil.

We report an exceedingly rare case of EGPA presenting with GBS-like symptoms in the absence of asthma, illustrating the remarkable clinical heterogeneity of this condition. Despite the absence of hallmark features, timely diagnosis was achieved through thorough systematic investigation, underscoring the critical importance of accurate diagnosis and prompt therapeutic intervention.