Chasing the Lymph: New Clues for the Management of Idiopathic Plastic Bronchitis

摘要

In this month's issue of AnnalsATS, Itkin and colleagues (pp. 1689-1696) provide strong evidence that at least some forms of plastic bronchitis, a rare syndrome characterized by the expectoration of branching bronchial casts, are caused by leakage of lymphatic fluid into central airways, associated with the result of occult abnormalities in the thoracic lymphatic system (1). The leaked fluid accumulates, thickens, and molds into rubbery casts that take on the branching shape of the airways (2). In the current article, the authors used dynamic contrast-enhanced magnetic resonance lymphangiography and intranodal lymphangiography to define abnormal thoracic lymphatic anatomy and pinpoint the source of the lymph leak in six adults with plastic bronchitis. They then demonstrated successful use of percutaneous lymphatic embolization to attenuate the lymph leak, resulting in partial or complete resolution of symptoms. The pioneering work by Itkin and coworkers represents a major advance not only in our understanding of the pathogenesis of some cases of plastic bronchitis, heretofore considered idiopathic, but also in developing effective treatment for this rare, debilitating, and sometimes life-threatening condition.