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Sjögren’s syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, causing xerostomia and keratoconjunctivitis sicca. Beyond glandular involvement, it can affect multiple organs, including the lungs, where it may present as lymphocytic interstitial pneumonia (LIP), amyloidosis, or rarely, lymphoma. Localized amyloidosis (AL type) results from deposition of monoclonal immunoglobulin light chains, while mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B-cell malignancy associated with chronic inflammation. The coexistence of pulmonary AL amyloidosis and MALT lymphoma in SS, particularly in a patient on long-term dialysis, is exceedingly rare. We report a case of a young woman with SS who developed pulmonary AL (κ-type) amyloidosis followed by disseminated MALT lymphoma identified at autopsy
A 34-year-old woman was diagnosed with SS based on renal and salivary gland biopsies showing interstitial nephritis and lymphocytic infiltration, together with positive anti-SS-A/SS-B antibodies. She was treated with prednisolone, mizoribine, and azathioprine but developed end-stage renal disease requiring hemodialysis ten years later.Seven years before death, she exhibited elevated immunoglobulins and an IgA-κ M-protein, diagnosed as monoclonal gammopathy of undetermined significance (MGUS). Three years before death, she developed progressive dyspnea, and chest CT revealed multiple pulmonary nodules. Bronchoscopy demonstrated lymphocytic infiltration without amyloid deposition or light-chain restriction. Tacrolimus therapy failed to improve her symptoms. A subsequent video-assisted thoracoscopic (VATS) lung biopsy identified AL (κ-type) amyloidosis by mass spectrometry. Systemic amyloidosis was excluded by bone marrow biopsy, cardiac MRI, and gastrointestinal evaluation. Melphalan and dexamethasone therapy were initiated for localized pulmonary amyloidosis, but respiratory failure gradually progressed, requiring home oxygen therapy.She later suffered a fatal intracerebral hemorrhage unrelated to coagulation disorders or hypoglycemia. An autopsy was performed to determine the cause of death and evaluate potential systemic amyloid involvement.
Autopsy revealed a massive left capsular cerebral hemorrhage with ventricular rupture, leading to herniation. There was no amyloid deposition in cerebral vessels, suggesting dialysis-related vascular fragility as the cause.Histologically, diffuse plasma cell proliferation with lymphoepithelial lesions and κ light-chain restriction was found in the gastric mucosa, confirming gastric MALT lymphoma. Similar lesions were observed in the lungs, esophagogastric junction, urinary bladder, and mesenteric lymph nodes, indicating disseminated MALT lymphoma. Amyloid deposition was limited to the lungs, confirming localized AL amyloidosis without systemic spread.
This case demonstrates the rare coexistence of pulmonary AL amyloidosis and disseminated MALT lymphoma in a patient with Sjögren’s syndrome receiving maintenance dialysis. Chronic antigenic stimulation in SS may drive monoclonal B-cell proliferation, leading to clonal κ light-chain production, localized amyloid deposition, and eventual malignant transformation.In SS, pulmonary amyloidosis typically presents as localized AL-type nodules, requiring histologic confirmation by thoracoscopic biopsy, as noninvasive tests are often inconclusive. MALT lymphoma occurs in approximately 4–8% of SS-related lymphomas and most frequently arises in the stomach, though endoscopic findings can be subtle and nonspecific.In this patient, MALT lymphoma was diagnosed only postmortem, indicating that earlier endoscopic surveillance might have enabled detection and management. The case highlights the need for continuous vigilance for lymphoproliferative complications in SS, particularly in patients with monoclonal gammopathy or pulmonary amyloidosis, and supports periodic gastrointestinal evaluation in long-term disease management.
This report expands understanding of the disease spectrum in SS, suggesting a continuum from chronic inflammation to monoclonal gammopathy, localized amyloid deposition, and eventual MALT lymphoma under the influence of autoimmune activity and dialysis-related immune dysregulation.
