Amiloidose traqueobrônquica primária

Abstract

A retired, hypertensive 67-year-old male, a former smoker and alcoholic, was referred to our facility with complaints of progressive dyspnea, wheezing and chest pain for the last 6 months. A chest X-ray obtained 8 years previously showed right hilar enlargement. He also had a long-standing history of episodic hemoptysis that ceased 10 years prior, together with a history of recurrent respiratory infections. Upon physical examination, he presented diminished breath sounds and slight crackles at the lung bases. A second chest X-ray revealed no changes in comparison with the first. The IntroductionThe amyloidoses constitute a group of diseases in which extracellular protein folding, or rather misfolding, plays a prominent role.(1) The disease can be hereditary or acquired and can be systemic or localized. Some patients are asymptomatic, whereas others exhibit a range of symptoms, and, in certain cases, the disorder can even be severe and life-threatening.(2) Tracheobronchial amyloidosis is an uncommon localized form of amyloidosis, characterized by amyloid deposits restricted to the trachea, main bronchi and segmental bronchi. AbstractTracheobronchial amyloidosis is an uncommon localized form of amyloidosis, characterized by amyloid deposits restricted to the trachea, main bronchi and segmental bronchi. We present the case of a retired 67-year-old man with long-term progressive dyspnea, wheezing and chest pain. A diagnosis of tracheobronchial amyloidosis was made after the third fiberoptic bronchoscopy and histological confirmation through Congo red staining of tissue samples.Keywords: Amyloidosis/trachea; Bronchoscopy; Tracheal diseases/diagnosis. ResumoA amiloidose traqueobrônquica é uma forma pouco comum de amiloidose localizada, caracterizada por depósitos amilóides limitados à traquéia, brônquios principais e brônquios segmentares. Nós apresentamos o caso de um homem aposentado de 67 anos com dispnéia progressiva de longa data, sibilância e dor torácica. O diagnóstico de amiloidose traqueobrônquica foi realizado após três fibrobroncoscopias e confirmação histopatológica com coloração vermelho congo.Descritores: Amiloidose/traquéia; Broncoscopia; Doenças da traquéia/diagnóstico.

Pulmonary amyloidosis with calcified nodules and masses - a six-year computed tomography follow-up: a case report

“…Tracheobronchial amyloidosis generally presents with symptoms of airway obstruction, such as dyspnea or cough, hemoptysis, and recurrent pneumonia [9,11]. Classic radiological signs of tracheobronchial disease include nodular and irregular narrowing of the tracheal lumen, airway wall thickening, and calcified amyloid deposits.…”

“…Histopathological diagnosis is made by the finding of amyloid, which is an inert, proteinaceous, homogeneous, acellular, and eosinophilic material that, when subjected to histochemical staining with Congo red shows green birefringence under polarized light [2,4,11-13]. This immunohistochemistry method remains the gold standard of diagnosis [12].…”

Tracheobronchial Amyloidosis Mimicking Tracheal Tumor

“…Tracheobronchial amyloidosis (TBA) is an uncommon localized form of amyloidosis, characterized by abnormal amyloid deposits in the trachea and main bronchi [5]. Amyloidosis commonly affects males (2 : 1) in middle age (50–60 years) groups [6].…”

A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis

“…Amyloid nodules are generally localised to the lower lobes, in the peripheral and subpleural areas [12]. They have four characteristic features on CT: sharp, lobulated contours, calcification, and often central or in an irregular pattern within the nodule (seen in about 50% of cases) [13, 14], with multiple shapes and sizes varying from 0.5 to 15 cm [14] and slow growth, often over years, with no regression [12]. Cavitation is very rare [15].…”