Multiple Pulmonary Nodules in a Patient with Sjögren's Syndrome: Extranodal Marginal Zone Lymphoma and Amyloidosis: A Case Report

Sjgren's syndrome (SS) is a chronic autoimmune disease characterized by systemic involvement that primarily affects the exocrine glands.Although pulmonary involvement is often asymptomatic, it can present with interstitial lung disease, airway abnormalities and, in rare cases, pulmonary amyloidosis and lymphoma.A 69-year-old female with a history of SS and a previous diagnosis of extranodal marginal zone B-cell lymphoma (EMZBCL) following a parotidectomy presented with pleuritic chest pain and unintentional weight loss.Imaging revealed multiple pulmonary nodules and ground-glass opacities, and the nodules were assessed to be at high risk for malignancy.A biopsy and segmentectomy decision was made based on a multidisciplinary evaluation of PET-CT findings, and the resulting pathology revealed EMBCL and widespread AL amyloidosis.Chemotherapy was subsequently planned.This case highlights that pulmonary involvement in SS can remain hidden, requiring the careful monitoring of pulmonary symptoms in recognition of the high malignancy risk.A multidisciplinary approach can play a critical role in the management of such complex cases.