Primary Bronchopulmonary Amyloidosis: A Report Of 2 Cases And Literature Review

Objective:To investigate the clinical manifestations,imaging features,diagnosis and treatment of primary bronchopulmonary amyloidosis,so as to improve diagnosis level,decrease misdiagnosis rate and enhance the prognosis.Method:A retrospective analysis of 399 cases was made,among which two cases we report and the clinical data of the other 397 cases were studied by literature from 1982 to 2018 in China.The analysis included demographic characteristics,clinical features,imaging findings,bronchoscopic manifestations,pathologies,treatments,and efcacy evaluation.Result:1.Primary bronchopulmonary amyloidosis is a kind of rare disease.So far,there were only 399 cases been reported in China,with a higher incidence rate of 1.6: 1 for men than for women.Their onset age was 11 to 81 years(mean 52.7 years)and the predilection age was 50 to 60 years old.The average age of onset in females was lower than that in males(P=0.015).2.Among the three types of primary bronchoalveolar amyloidosis,primary tracheobronchial amyloidosis was most common(85.8%),and the atmospheric tract was the most common site.3.The clinical manifestations of the disease are lack of specificity.Their main clinical manifestations are Cough,expectoration,hemoptysis,blood in phlegm,shortness of breath,shortness of breath,dyspnea,fever and hoarseness.But the most common is cough,especially dry cough.4.The positive staining of Congo red was the golden standard for the diagnosis of the disease in histopathology.The biopsy under bronchoscope is the most commonly which used in 80% of patients.The other methods include that underwent CT guided percutaneous lung biopsy,fiberoptic laryngoscopy biopsy and autopsy.5.The laboratory examination lacks the specificity,and the examination result of the disease mostly has no abnormality.Obstructive ventilation dysfunction was the main manifestation of pulmonary function examination,and mild ventilation was the most common.Chest CT examination is the most commonly used in imaging examination.Irregular stenosis of the wall is the most common imaging manifestation of bronchopulmonary amyloidosis(69.9%).We can see different forms of calcification in the lesion.Bronchoscopy is the main method of diagnosis and treatment of this disease,the most common manifestation we can see of airway wall thickening and lumen stenosis under endoscopy(85.8%).6.Patients with primary bronchopulmonary amyloidosis are often misdiag-nosed as pulmonary tuberculosis,lung cancer,chronic bronchitis,etc.Of these,tuberculosis is most likely to be misdiagnosed(10.5%).7.The main treatments for this disease are as follows: drug therapy,bronchoscopic interventional therapy,surgical resection,external chest irradiation(EBRT),and the other options: autologous stem cell transplantation,hard endoscope resection.Multiple treatment regimens are commonly used in clinical practice,and most of them can achieve better short-term results.The curative effect of bronchial endoscopy combined with drug therapy is better than that of bronchial interventional therapy alone(P=0.005).8.The patients with primary bronchopulmonary amyloidosis have low mortality and long survival time.Conclusion:Primary bronchopulmonary amyloidosis is a rare respiratory disease,the pathogenesis of which is unknown.The incidence of the disease is higher in men than in women.There was no specificity in clinical manifestations and laboratory tests.Histopathological examination is the golden standard for diagnosis of it.According to pathology,it is divided into three types,the most common type of tracheobronchial amyloidosis.Up to now there is no standardized treatment,tracheoscopy is the main means of diagnosis and treatment of this disease,bronchoscopic interventional therapy can improve airway stenosis,and rapid relief of symptoms.The treatment bronchoscopic interventions combined with chemotherapy is better than using alone.