Clinical features include exertional dyspnea, cough, chest pain, recurrent pneumothorax, chylous pleural effusion, hemoptysis, eventual respiratory failure and, ultimately, death.

• 临床表现有呼吸困难、胸痛、咳嗽、咳血、反覆性气胸、乳糜胸，甚至呼吸衰竭，进而死亡。