Allergic Bronchopulmonary Aspergillosis in Patients with Dominant Negative IL6ST

Pathogenic variants in IL6ST cause a Hyper-IgE syndrome (HIES) that presents similarly to STAT3 dominant negative (DN) HIES. Severe structural lung disease with bronchiectasis and pneumatoceles is a major driver of morbidity in this disease. We describe clinical characteristics of four patients with heterozygous IL6ST variants with presentations consistent with allergic bronchopulmonary aspergillosis (ABPA).