CTICasesLymphoid Interstitial Pneumonia (LIP) with Concurrent MALT Lymphoma in Sjogren's Syndrome
Imaging findings
CT in a patient with Sjogren's syndrome demonstrates bilateral cystic lung disease with scattered soft tissue nodules consistent with LIP, including lymphoid aggregates and amyloid. A dominant nodule in the right middle lobe has grown steadily on serial imaging. This nodule was resected and confirmed to be MALT lymphoma. A subsequent CT shows an additional growing nodule in the left lower lobe, concerning for multifocal MALT lymphoma in the setting of LIP and Sjogren's syndrome.
Key takeaways
Sjogren's syndrome is associated with a spectrum of lymphoproliferative lung disease ranging from LIP (polyclonal lymphoid proliferation producing cystic lung disease and nodules) to MALT lymphoma (monoclonal low-grade B-cell lymphoma). These conditions co-exist and any growing or dominant nodule in a patient with Sjogren's and LIP should raise concern for development of lymphoma. LIP is not a premalignant precursor per se, but patients with Sjogren's have an underlying tendency toward lymphoproliferation that independently predisposes to lymphoma. Close surveillance of nodule growth in Sjogren's LIP patients is essential.
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