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Clinical, Biomarker, and Radiological Progression from Asthma to Systemic Eosinophilic Granulomatosis with
Chenyang Lu1, Changxing Ou1, Yu Deng2
1State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, National Center for Respiratory Medicine, Department of Pulmonary and Critical Care Medicine, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, People's Republic of China.
Background:
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-organ autoimmune disease characterized by eosinophilic infiltration of peripheral blood and tissues, and necrotizing granulomatous inflammation of small and medium-sized blood vessels. In the prodromal stage of EGPA, patients may present with features of refractory asthma, with the involvement of other organs occurring later when the diagnosis of EGPA is made. The difficulty of early diagnosis makes treatment difficult.
Methods:
We retrospectively describe patients (N=13) who attended the asthma clinic at the First Affiliated Hospital of Guangzhou Medical University between 2008 and 2024. The disease course was categorized into three stages: asthma, lung-limited or lung-dominant EGPA (L-EGPA), and systemic EGPA (S-EGPA). Patients with severe eosinophilic asthma served as controls. We evaluated baseline demographic, as well as organ involvement, complication, laboratory findings, lung function, high-resolution computed tomography (HRCT), and treatment across different disease stages. A case-crossover design and Bayesian conditional logistic regression were employed to evaluate the impact of medication use on disease progression.
Results:
We identified a group of EGPA patients who exhibited consistent disease progression to transit from asthma to L-EGPA, and eventually to S-EGPA. These stages exhibit distinct clinical and imaging features, with significantly elevated eosinophilic inflammatory markers in induced sputum or blood being a hallmark of L-EGPA. This distinction may aid in differentiating refractory asthma from L-EGPA.
Conclusion:
In conclusion, the L-EGPA phase may represent a distinct stage in EGPA development that is often challenging to distinguish from refractory asthma. Characterizing this phase and identifying specific biomarkers could facilitate earlier diagnosis and treatment, potentially improving patient outcomes-a hypothesis that warrants further validation.
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