Abstract
BACKGROUND: Sjogren's syndrome (SS), which affect salivary gland function, is an autoimmune disease. SS may involve extraglandular organs. Approximately 10 to 20 percent of SS patients have clinically significant lung disease, but presentation of pulmonary amylodosis is extremly rare. The incidence of benign monoclonal gammopathy in SS patients is high, but multiple myeloma is rare. No case involving the simultaneous occurrence of two rare diseases, pulmonary amyloidosis and multiple myeloma, in the same patient with SS has been reported so far. CASE SUMMARY: A 41-year-old male patient was referred to our hematology department due to incidentally detected gastric plasmacytoma. He had been diagnosed with SS four years earlier. Multiple miliary nodules, ground glass opacity in both lung fields, and enlargement of both inguinal lymph nodes was observed on chest and abdomen computer tomography. Based on the pathological findings of lung and lymph node biopsied specimens, the patient was diagnosed with pulmonary amyloidosis and multiple myeloma. Pulmonary amyloidosis and multiple myeloma associated with SS has rarely been reported. CONCLUSION: This is an extremely rare case of simultaneous pulmonary amyloidosis and multiple myeloma in the same patient with SS.