CTICasesTracheobronchial MALT Lymphoma in Sjogren Syndrome
Imaging findings
CT chest and bronchoscopy in a 77-year-old female with Sjögren syndrome and basilar-predominant fibrotic NSIP show smooth, broad-based submucosal soft tissue masses causing narrowing of the right main bronchus and left main bronchus. Immunohistochemistry of the biopsy specimen confirmed a monoclonal B-cell marginal zone lymphoma (MALT lymphoma) with Kappa light chain restriction.
Key takeaways
Sjögren syndrome is an autoimmune disease characterized by chronic lymphoepithelial lesions and a significantly increased risk (up to 44-fold) of developing B-cell non-Hodgkin lymphoma, particularly marginal zone B-cell lymphoma (MALT lymphoma). While pulmonary MALT lymphoma typically presents as nodules or consolidation, it can rarely manifest as submucosal airway masses.
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