This study presents a rare case of pulmonary amyloidosis and cystic lung disease associated with primary Sjögren's syndrome. In February 2019, a 76-year-old woman with primary Sjögren's syndrome presented to our rheumatology department for lung problems. Four years before presentation, the patient underwent chest computed tomography that revealed multiple nodules with numerous thin-walled lumen in both lungs. Pulmonary nodulectomy for hemoptysis was performed 2 years before presentation. Pathological findings suggested chronic inflammation of the bronchus and amyloid deposition in the lung tissue. The nodules and vesicles were generally larger from January 2015 to October 2018 when she did not receive any special medication, but the nodules and vesicles tended to be stable from February to August 2019 when she was treated with hydroxychloroquine sulfate regularly, suggesting that hydroxychloroquine sulfate may be helpful in this patient.
Keywords: Cystic lung disease; Hemoptysis; Hydroxychloroquine sulfate; Primary Sjögren’s syndrome; Pulmonary amyloidosis.
© 2021. International League of Associations for Rheumatology (ILAR).