Pulmonary alveolar proteinosis as an unusual pattern of lung involvement in Sjögren syndrome.

Primary Sjögren syndrome (pSS) is an autoimmune disease that is characterized by infiltration of lymphocytes in exocrine glands, followed by hypofunction of the glands. Dry mouth and eyes are main symptoms, but there are various extraglandular manifestations in pSS. Nine to 75% of patients have pulmonary involvement such as interstitial lung diseases. Pulmonary alveolar proteinosis (PAP) is also a kind of autoimmune disorder, characterized by the presence of anti-granulocyte/macrophage colony-stimulating factor antibody. A 45-year-old nonsmoking woman was admitted with dyspnea and dry cough. She was diagnosed as having PAP, based on characteristic computed tomography, bronchoalveolar lavage, and lung biopsy findings. At the same time, she was diagnosed with pSS, based on dry mouth, xerophthalmia, positive anti-Ro/La antibodies, and scintigraphic finding of salivary glands. Whole lung lavages and monthly intravenous cyclophosphamide had slight improvement in PAP.