Management of tracheobronchial amyloidosis: a review of the literature

Abstract

IntroductionTracheobronchial amyloidosis (TBA) is a rare idiopathic disorder characterized by extracellular deposition of misfolded protein fibrils in the tracheobronchial tree. It presents with non-specific symptoms. Deciding on the best treatment approach can be challenging due to the lack of a treatment guideline. We undertook a review to review the therapeutic options for tracheobronchial amyloidosis and to highlight gaps within the existing evidence.MethodsWe performed a literature search from 1st January 1990 until 1st March 2022 to identify relevant literature regarding patient characteristics, symptoms, management and prognosis for patients with TBA.ResultsA total of 77 studies consisting of 300 patients were included. We found a great heterogeneity in the management of TBA patients. Although a fifth of the reported patients were managed with a wait and see approach many different treatments were used as a single intervention or multiple treatments were combined. An interesting finding is the slightly higher percentage of patients with Morbus Sjögren (n=5, 1.7%) and TBA compared to the normal population (0.5% to 1.0%).ConclusionsThere is a great heterogeneity in the management of TBA patients. The treatment is still based on expert opinion due to the lack of a treatment guideline. Various treatment approaches include a wait and see approach, external beam radiotherapy, therapeutic bronchoscopy, immunosuppressive treatment and surgery.