We report a 58-year-old female patient with Sjögren’s syndrome (SS) and amyloidosis who presented with chronic cough. Her initial chest roentgenograph showed multiple bilateral nodules, linear infiltrates and cysts. Her condition remained stable for three years, but then deteriorated progressively a few months after beginning levamisole treatment. Multiple pulmonary bullae were recognized concurrently. A open lung biopsy disclosed nodular amyloidosis and a low-grade malignant lymphoma. SS associated with amyloidosis and pulmonary bullae formation has rarely been reported. We also believe it is the first report of the effects of levamisole in such a condition. A determination of the possible detrimental effects of levamisole may require further study.