[Two patients with a rare manifestation of amyloidosis in the respiratory system].
Amyloidosis of the respiratory tract was diagnosed in 2 patients. The patients were men, 62 and 55 years ofage. The first patient presented with dyspnoea and diffuse parenchymal lung abnormalities. There was a rapidly progressive obstructive lung function disorder and a severe diffusion impairment. The second patient had haemoptysis due to tracheobronchial amyloidosis. Amyloidosis of the respiratory system is rarely diagnosed. Nearly all cases ofclinically relevant respiratory amyloidosis are due to light chain amyloid (AL amyloidosis). The described diffuse lung parenchymal abnormalities are a manifestation of systemic AL amyloidosis. On the other hand, tracheobronchial amyloidosis is a disorder which usually remains localised in the airways. Systemic AL amyloidosis may be treated with chemotherapy or stem cell transplantation. It is unknown whether this treatment leads to a decrease of pulmonary function abnormalities. Tracheobronchial amyloidosis can be treated by endobronchial therapy. Often this treatment is required repeatedly. The first patient died 2 months after diagnosis due to pneumonia. The second patient was treated with endobronchial argon plasma coagulation and diathermy and has been symptom-free for 3 years since.
Jacobs
W
W
VU Medisch Centrum, Postbus 7057, 1007 MB Amsterdam. w.jacobs@vumc.nl
Vonk Noordegraaf
A
A
Sutedja
T G
TG
Grünberg
K
K
Postmus
P E
PE
D000402:Airway Obstruction / Q000453:epidemiology / Q000209:etiology; D000686:Amyloidosis / Q000150:complications* / Q000453:epidemiology; D001982:Bronchial Diseases / Q000453:epidemiology / Q000209:etiology*; D017024:Chemotherapy, Adjuvant / Q000379:methods; D017809:Fatal Outcome; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016896:Treatment Outcome