CTICasesPulmonary Amyloidosis and Marginal Zone Lymphoma in Sjogren's Syndrome
Imaging findings
High-resolution chest CT demonstrates bilateral, progressive, irregular interstitial thickening, subpleural reticulation, and multiple pulmonary nodules. Some nodules contain calcified foci. Biopsy of a nodule demonstrates AL amyloid deposition and clonal plasma cell infiltrates consistent with marginal zone lymphoma.
Key takeaways
Sjogren's syndrome is associated with an increased risk of lymphoproliferative disorders, including marginal zone lymphoma (MALT lymphoma). These lymphomas can produce monoclonal light chains, leading to localized AL amyloid deposition in the lung parenchyma, which can manifest as calcified nodules and cysts. The prognosis and treatment depend on both the lymphoma and systemic amyloid involvement.
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