CHEST 2023 On Demand Pass-Comprehensive Approach to Cystic Lung Diseases

Video Transcription

Video Summary

During this session, three cases of diffuse cystic lung disease were presented. In the first case, a 35-year-old woman with a family history of neurofibromatosis presented with cysts on CT imaging and was diagnosed with sporadic lymphangioleiomyomatosis (LAM) based on high levels of HTLV and negative alpha-1 antitrypsin levels. The management of LAM includes patient education on pneumothorax and vaccination, smoking cessation, and treatment with mTOR inhibitors if lung function is declining or there are other indications. In the second case, a 50-year-old woman presented with dyspnea and nodular opacities on chest CT. She was eventually diagnosed with pulmonary Langerhans cell histiocytosis (LCH) based on characteristic CT findings and confirmed with a biopsy showing CD1A positive cells. The management of LCH includes smoking cessation and treatment with cladribine or Vemurafenib in some cases. In the third case, a 35-year-old woman with acute chest pain was found to have cysts on CT and was eventually diagnosed with sporadic LAM based on high levels of VEGF-D, a family history of neurofibromatosis, and genetic counseling. The management of sporadic LAM includes patient education on pneumothorax and vaccination and treatment with serolimus if lung function is declining or there are other indications.

Meta Tag

Category Diffuse Lung Disease

Session ID 1068

Speaker Misbah Baqir

Speaker Jonathan Chung

Speaker Jean Paul Higuero Sevilla

Speaker Rachana Krishna

Track Diffuse Lung Disease

Keywords

diffuse cystic lung disease

sporadic lymphangioleiomyomatosis

LAM management

pulmonary Langerhans cell histiocytosis

LCH management

cladribine

Vemurafenib

VEGF-D

serolimus