[STAT3 gene-related hyper-IgE syndrome complicated by pulmonary aspergillosis: a case report]

Patients with signal transducer and activator of transcription 3-related hyper-IgE syndrome (STAT3-HIES) complicated by invasive pulmonary aspergillosis typically show rapid disease progression, a tendency toward systemic dissemination, prolonged treatment courses, frequent relapse after therapy, and a poor prognosis. Some patients with STAT3-HIES also develop allergic bronchopulmonary aspergillosis (ABPA). Conventional treatment of ABPA relies primarily on glucocorticoids; however, because patients with STAT3-HIES already have immune deficiency, glucocorticoid therapy may further suppress immune function. New therapeutic strategies using biologic agents for STAT3-HIES complicated by ABPA are urgently needed. We report a patient with STAT3-HIES complicated by invasive pulmonary aspergillosis and ABPA who was treated with oral posaconazole, bronchoscopic instillation of amphotericin B, and omalizumab. Afte treatment, the patient's cough was alleviated, pulmonary function improved, mucus plugs resolved, and serum total IgE decreased significantly. By summarizing this case together with a literature review, we hope to raise awareness of STAT3-HIES complicated by invasive pulmonary aspergillosis and ABPA and to improve future treatment strategies.