SESSION TYPE: Airway Global Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: A 38 years old man presented a chronic upper right lobe atelectasis complicated by recent infection. After completing diagnostic workup, excluding infection, autoinmune disease and neoplasm, an unexpected diagnosis of tracheobronchial amyloidosis came up.
CASE PRESENTATION: A 38 year old man was admitted to hospital with a 72 hours of fever (38 C), left chest pain and dyspnea MRC 3. He was mild hypoxemic, a right upper lobe atelectasis and a left basal consolidation was evident on the chest X Ray. By patient referral the right atelectasis was present since 3 years, and had undergone two previous bronchoscopies in another institution without diagnostic yield. Antibiotic therapy was established with ressolution of the left consolidation and persistance of the right atelectasis. Thoracic CT and Bronchoscopy were performed. On CT: Concentric and diffuse thickening of trachea, main , segmental and subsegmental bronchi walls. Upper right lobe bronchial stenosis with total lobe atelectasis. Bronchoscopy: right upper lobe bronchus occupied by tumor-like formation that was biopsed. Lateral basal segment of left lower lobe with a thickened lobar carina with infiltrative appearance. Biopsy showed: Deposit of amorphous eosinophilic hyaline material which stained with Congo red and refracted under polarized light greenish yellow. An Amyloidosis diagnosis was confirmed. Immunohistochemistry was consistent with AA amyloidosis. No other systemic compromise was found.
DISCUSSION: Tracheobronchial amyloidosis is an uncommon diagnosis. Airway involvement may be focal, multifocal, or diffuse. Diffuse involvement is most common as in this case (1). Affected patients are often asymptomatic for a long time before diagnosis, which suggests that the disease progresses relatively slowly. Narrowing of the airways can cause distal atelecatasis or recurrent pneumonia (2). Endoscopic examination of the disease shows either submucosal plaques and nodules with a cobblestone appearance or a tumor-like appearance or circumferential wall thickening. Endoscopic biopsies are diagnostic. Deposition of amyloid in the tracheal bronchi may be seen in association with systemic amyloidosis or as an isolated manifestation.
CONCLUSIONS: Nonneoplasic central airway disease must be considered as a cause of chronic atelectasis. Tracheobronchial amyloidosis has inespecific clinical symptoms. Chest CT helps in diagnostic suspicious. Biopsy confirms diagnosis.
1) Nonneoplastic Tracheal and Bronchial Stenoses. Philippe A. Grenier, Catherine Beigelman-Aubry, Pierre-Yves Brillet. PhDb Radiol Clin N Am: 47 (2009) 243-260.
2) Amyloidosis and the respiratory tract .Julian D Gillmore, Philip N Hawkins,Thorax 1999;54:444-451
DISCLOSURE: The following authors have nothing to disclose: Patricia Malamud, Eduardo Giugno, Valeria Morandi, Nicolas Itcovici, Santiago Rossi, Andrea Werbach
No Product/Research Disclosure InformationHospital Dr. Antonio A. Cetrángolo, Buenos Aires, Argentina