Tracheobronchial Amyloidosis:Report Of 3 Cases And Literature Review

Amyloidosis is a clinical syndrome caused by misfolding of autologous protein and its extracellular deposition as fibrils,resulting in vital organ dysfunction and eventually death.Pulmonary amyloidosis is a rare disease.According to the definition of the world health organization(WHO),the number of patients with rare diseases accounts for 0.65‰-1 ‰ of the total population.The annual incidence of pulmonary amyloidosis was 0.01‰ according to figures.Tracheobronchial amyloidosis was more rare and with no specific symptoms.The main clinical symptoms included cough,chest distress,dyspnea,hemoptysis,and so on.The imaging manifestations included calcification(which was specific),thickening of wall,and narrowing of the lumen.In enhanced scanning,strengthened lesions could be observed.Clinical misdiagnosis and missed diagnosis often occurred due to localized tracheobronchial amyloidosis.Diffused lesions with hilar lymph node enlargement,calcification,and pulmonary nodular or patchy high-density shadow were often misdiagnosed as bronchial asthma,tuberculosis,chronic bronchitis and other diseases.Misdiagnosis and missed diagnosis frequently occur due to insufficient clinical and imaging understanding of the disease.Comprehensive analysis of the imaging manifestations and clinical characteristics,together with pathological examination,can help avoid possible misdiagnosis.The clinical data of 3 cases of tracheobronchial amyloidosis were retrospectively analyzed,the end were tracked,and relevant literature of missed cases were reviewed,and also pathogenesis,clinical manifestations,diagnosis,staging and treatment of respiratory amyloidosis.In this study,the age and sex of the 3 cases were:72(male);75(male)55;(female),all of which were pathologically confirmed by bronchoscopy with primary airway or bronchial stenosis.Patients with amyloidosis had a high risk of bleeding,and the lesions were prone to bleeding during the examination.No interventional treatment was given.Hormone therapy was given after diagnosis.After treatment,the patient was released from the hospital.After follow-up,the symptoms did not worsen repeatedly,and the therapeutic effect was satisfactory.Amyloidosis of the respiratory tract is rare,but may represent a significant clinical problem in either systemic or organ-limited amyloidosis,and Clinical misdiagnosis and missed diagnosis often occurred.Pathological diagnosis is the gold standard,The improvement of biopsy of the site of scathing or other easily obtained(such as subcutaneous fat,bone marrow,salivary glands)is conducive to the diagnosis and treatment.Most cases are asymptomatic and need only a careful follow-up.The treatment of diffuse alveolar septal amyloidosis is usually chemotherapy or hematopoietic stem cell transplantation.Nodular pulmonary amyloidosis is usually localised,conservative excision is usually curative and the long-term prognosis is excellent.Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.In addition to the traditional chemotherapy drugs such as Mafalda,cyclophosphamide,etc,proteasome inhibitors(PI)such as bortezomib,carfezomib,ishazomib have been used in clinical treatment,Monoclonal antibodies such as radar monoclonal antibodies have entered phase ? trials in patients with amyloidosis,the phase ? VITAL trial of Serum amyloid P component(SAP)was stopped due to futility.