Amyloid-associated cystic lung disease in a woman with Sjögren syndrome

A 68-year-old Caucasian female was admitted for a traumatic subarachnoid hemorrhage with bilateral subdural hematoma. The patient underwent a total body computed tomography scan that revealed the presence of a cystic lung disease associated with bilateral, partly calcified, lung nodules. She denied any respiratory symptoms but described mild xerophthalmia. A positivity for antinuclear antibodies and extractable nuclear antigen antibodies (i.e., anti-SSA/Ro-52) was detected, and a diagnosis of Sjögren syndrome was made. The patient initially refused to undergo a pulmonary biopsy, but following a dimensional increase of a lung nodule after 2 years from the baseline assessment, a video-assisted thoracoscopic biopsy was performed. The histopathological evaluation showed the presence of pulmonary nodular k light-chain amyloidosis. A final diagnosis of amyloid-associated cystic lung disease in a patient with Sjögren syndrome was made.