Eosinophilic Granulomatosis with Polyangiitis Disguised in a Patient with Episodic Wheezing for Thirteen Years: A Case Report

Vollständiger Abstract

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare primary small-vessel vasculitis. Diagnosis of EGPA is challenging in the early stage, because the disease is limited to the respiratory system. Similar clinical features are observed in other eosinophilic lung diseases, such as bronchial asthma, allergic bronchopulmonary aspergillosis, and eosinophilic syndrome. Careful clinical follow-up is necessary to detect disease behaviour and support diagnosis. We report a 53-year-old female who has had long-standing episodic wheeze and difficulty in breathing for 13 years. The initial evaluation revealed severe eosinophilia with a positive Aspergillus antigen skin test and was managed as presumptive allergic bronchopulmonary aspergillosis (ABPA). Remarkable clinical improvement with corticosteroids led to corticosteroid dependence. Subsequent development of peripheral polyneuropathy and biopsy-proven eosinophilic vasculitic cutaneous eruption led to the diagnosis of EGPA after thirteen years of initial presentation despite a negative perinuclear anti-neutrophilic cytoplasmic antibody (p-ANCA) test. This case highlights the importance of clinical vigilance and careful follow-up in patients with eosinophilic chronic lung disease.