[A case of multiple nodular pulmonary amyloidosis associated with Sjögren's syndrome]

A 58-year-old female, who had complained of dryness of the conjunctiva and mouth for nine years, was admitted to our hospital because chest X-ray films revealed increases in both the size and the number of pulmonary nodules in comparison to four years earlier. Histological findings of the salivary gland biopsy confirmed a diagnosis of Sjögren's syndrome. An open lung biopsy was performed. Histologically, the pulmonary nodules consisted of homogeneous, acellular eosinophilic materials, which were identified as amyloid by Congo red staining. Immunohistochemical examination showed the amyloid to be composed of AA protein. There were no deposits in other organs. Peribronchial lymphocytic infiltration associated with Sjögren's syndrome was present. Nodular pulmonary amyloidosis with Sjögren's syndrome is very rare condition. To our knowledge, this case is only the ninth to be reported in the literature.