Clinical analysis of bronchopulmonary amyloidosis secondary to primary Sj(O)gren's syndrome / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical characteristics of bronchopulmonary amyloidosis secondmy to primary Sj(0)gren's syndrome (pSS).Methods One patient in our series and 42 patients in the literatures were analyzed.The clinical manifestations,imaging features,diagnosis,treatments and prognosis of these patients were described respectively.Results Among the 43 cases,42 patients were female (98％).The median age was 57 (range 29-79) years.The diagnosis of bronchopulmonary amyloidosis was made subsequently to that of pSS with a median delay of 8.9 (range 0-30) years.Thirty-eight cases (88％) were localized amyloidosis.Most cases were related to AL amyloidosis (21/28,75％ ).The main clinical manifestations included cough (18/38,47％),short of breath (13/38,34％ ),sputum (9/38,24％) and hemoptysis (5/38,13％ ).Nine patients (9/38,24％ ) had no clinical symptoms.The most common patterns of radiological manifestations included multiple nodules (40/43,93％),multiple cysts or bullae (16/43,37％),interstitial lung disease (16/43,37％ ),irregular luminal narrowing and airway wall thickening (8/43,19％ ).The pulmonary function test was done in 17 patients,which revealed moderate to severe reduced diffusion capacity for carbon monoxide (8/17,47％ ).The diagnosis of amyloidosis was made based on pathological findings in all cases.Pathologic examination showed diffuse deposits of amorphous,eosinophilic,Congo-red positive staining material.The treatments were symptomatic.The prognosis of most patients was good.The median follow-up time was 26.5 (range 2-96) months,only 2 patients died during the follow-up.Conclusion The bronchopulmonary amyloidosis secondary to pSS is localized amyloidosis in most cases.Clinical manifestations depend on the location and extent of airway lesions.The appearance of multiple lung nodules with calcified or cysts in chest images should be considered as secondary amyloidosis.No specific therapy is available for these cases,however,this condition in majority of patients progresses slowly.