Primary tracheobronchial amyloidosis: a series of 3 cases.
Primary tracheobronchial amyloidosis is a form of localized pulmonary amyloidosis, characterised by the deposition of AL-amyloid in trachea and bronchi. It is a rare and slowly progressive disease, usually requiring repeated endoscopic treatment. In this case series we describe symptoms, diagnostic and therapeutic procedures, radiological findings and pulmonary function testing in 3 cases of different presentation and severity. Two patients were treated by endoscopic debulking and stent placement during rigid bronchoscopy, both with excellent clinical and functional results. In one of these patients regular endoscopic and clinical control exams were performed in the 5 years following the initial treatment, showing stable disease, requiring no further therapeutic intervention until today.
Hanon
S
S
Dienst pneumologie, Kliniek voor interventionele endoscopie, Universitair Ziekenhuis Brussel Laarbeeklaan 101, 1090 Brussel, België. shane.hanon@uzbrussel.be
De Keukeleire
T
T
Dieriks
B
B
Bultynck
W
W
Vanmaele
L
L
Meysman
M
M
Noppen
M
M
Vincken
W
W
D000328:Adult; D000686:Amyloidosis / Q000175:diagnosis* / Q000601:surgery; D001706:Biopsy; D001982:Bronchial Diseases / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D001999:Bronchoscopy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D019919:Prosthesis Implantation / Q000295:instrumentation; D013902:Radiography, Thoracic; D015607:Stents; D014057:Tomography, X-Ray Computed; D014133:Tracheal Diseases / Q000150:complications / Q000175:diagnosis* / Q000601:surgery