Amyloidosis and the Lungs and Airways

Abstract

Amyloidosis can both complicate long-standing respiratory conditions and be deposited within the respiratory system itself. In localised amyloidosis management generally involves resection of symptomatic deposits. In acquired systemic amyloidosis treatment is controlling the underlying condition.

Pulmonary Amyloidoma: A Case Report and Brief Review of the Literature

“…Additional hypotheses included fungal infections, benign nodules of various etiologies, neoplastic substrate, or, given that our country remains endemic for tuberculosis, a bacillary etiology. Similar to other reported cases, excisional surgical biopsy was mandatory, as the 'gold standard' for diagnosis continues to be the histopathological examination, which demonstrates apple-green birefringence with polarized microscopy after staining with Congo red [2]. The particularity of the case lies in the earliness of the diagnosis, given that the disease was detected in this limited form, with the absence of signs of systemic involvement, although it is not excluded that it may evolve into multi-organ impairment in the future.…”

“…In cases of localized amyloidosis, respiratory symptoms can range from asymptomatic pulmonary nodules to diffuse parenchymal deposits. As in our case, the clinical presentation is often characterized by cough, signs of bronchial obstruction, recurrent lower respiratory tract infections, associating symptoms such as dyspnea or wheezing, which can mimic other pulmonary diseases [2,7,9].…”

“…Amyloidosis can be extremely heterogeneous and describes a group of diseases characterized by the extracellular deposition of insoluble fibrillar proteins that can originate from serum proteins or be produced locally [2]. An important feature of amyloid proteins is their capacity to infiltrate almost all organs and systems within the body.…”

“…Since these proteins are deposited extracellularly in the form of amyloid fibrils and cannot be metabolized, they can induce both systemic and localized toxicity [3]. This leads to cell death, subsequent functional impairments, and gradual disruption of the structure, integrity, and function of the tissues [2,4].…”

“…Depending on the anatomic site of involvement [8], three histopathological patterns are recognized: diffuse alveolar-septal, nodular, and tracheobronchial [4,6,7]. Other classifications have been proposed based on radiographic or bronchoscopic findings [2].…”