Abstract

Involvement of the respiratory tract by amyloidosis, particularly amyloidosis that solely affects the respiratory organs, is a rare disease. This may result in difficulties with its recognition and diagnosis in daily routine, and therefore, amyloidosis of the respiratory tract might deserve renewed introduction. Analyses have suggested that in most situations, respiratory amyloidosis belongs to the AL type, whereas involvement of the respiratory organs is not prominent in either reactive AA amyloidosis or senile TTR amyloidosis. The main forms of respiratory amyloidosis include laryngeal amyloidosis, tracheobronchial amyloidosis, parenchymal amyloidosis, and mediastinal and hilar amyloidosis. The diagnosis of amyloidosis requires histological confirmation with Congo red method, however, an appropriate clinical approach to respiratory amyloidosis assumes chest X-ray, CT-scan, fiberoptic bronchoscopy, testing of lung function, as well as relevant tests and investigations to rule out dysfunction of the extrapulmonary organs. Due to the resemblance of the bronchoscopic picture of tracheobronchial amyloidosis to that of bronchogenic carcinoma, the former is often preliminarily misdiagnosed as cancer; a respective case is presented in this article.
Management of amyloidosis of the respiratory tract should be strictly individualized.
Symptomatic localized amyloidosis is usually amenable to intermittent bronchoscopic resection, surgical resection, carbon dioxide laser ablation, or Nd:YAG laser therapy, whereas systemic forms require systemic chemotherapy, usually with melphalan and prednisolone. Preliminary results with so-called “anti-amyloid” agents are also promising to control amyloid deposits. Patients with diagnosed respiratory amyloidosis should be followed up on a regular basis (approximately twice annually) with repeated chest X-ray, CT-scan, testing of lung function and exercise tolerance, and fibre optic bronchoscopy, in addition to routine blood tests, as well as urinalysis, serum biochemistry, electrocardiography and echocardiography to monitor organs’ dysfunction due to possible amyloid load.