Unicentric Castleman disease complicated with bronchiolitis obliterans: A single-centre retrospective study from...

Bronchiolitis obliterans (BO) is a rare and life-threatening complication of unicentric Castleman disease (UCD), which is considered the leading cause of death in UCD. However, few studies focused on the characteristics of UCD patients complicated with BO. This single-centre, retrospective study of 281 patients diagnosed with UCD identified 18 patients (6.4%) with BO. UCD-BO patients had a significantly lower age at UCD diagnosis than the UCD without BO group. 94.4% of UCD-BO patients had deep lymph nodes involvement and 83.3% of them had paraneoplastic pemphigus, which were both significantly higher than UCD without BO. Median forced expiratory volume in 1 s of predicted was 20.9% (10%-41.9%) and 83.3% patients had severe BO. All patients underwent complete resection for UCD. 61.1% patients developed BO after surgery. UCD-BO patients had a poorer overall survival than UCD without BO. Among severe BO, 11 patients did not receive lung transplantation and two of them died, with 62.5% (95% CI, 20.8%-100%) of estimated 5-year overall survival. Four patients underwent lung transplantation and they all survived. UCD-BO patients had distinct clinical identity. Complete resection could neither alleviate BO nor prevent BO progression. Lung transplantation is a vital treatment for improving prognosis for patients with severe BO.