Abstract
Sjögren syndrome (SS) is characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands, leading to keratoconjunctivitis sicca and xerostomia. SS is one of the most common autoimmune rheumatic diseases in adults; however, few cases of primary childhood SS with gastrointestinal and liver lesions have been reported in the literature. We report five cases of primary childhood SS with gastrointestinal and liver lesions. Multiple gastric biopsies in four cases revealed atrophic gastritis in the antrum of the stomach or chronic gastritis. Liver biopsies in two cases revealed nonalcoholic steatohepatitis. Careful clinical approach and follow-up for gastrointestinal and liver lesions are required.
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Abbreviations
- SS:
-
Sjögren syndrome
- EGM:
-
Extraglandular manifestation
References
Houghton K, Malleson P, Cabral D, Petty R, Tucker L (2005) Primary Sjogren’s syndrome in children and adolescents: are proposed diagnostic criteria applicable? J Rheumatol 32:2225–2232
Ienopoli S, Carsons SE (2014) Extraglandular manifestations of primary Sjögren’s syndrome. Oral Maxillofac Surg Clin North Am 26:91–99
Tsianos EV, Moutsopoulos HM (1989) Sjögren’s syndrome and the gut. Baillieres Clin Rheumatol 3:357–370
Sheikh SH, Shaw-Stiffel TA (1995) The gastrointestinal manifestations of Sjögren's syndrome. Am J Gastroenterol 90:9–14
Ebert EC (2012) Gastrointestinal and hepatic manifestations of Sjogren syndrome. J Clin Gastroenterol 46:25–30
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM et al (2017) 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 76:9–16
Koskenpato K, Ainola M, Przybyla B, Kouri VP, Virkki L, Koskenpato J, Ristimäki A, Konttinen YT (2016) Diminished salivary epidermal growth factor secretion: a link between Sjögren's syndrome and autoimmunegastritis? Scand J Rheumatol 45:118–121
Hasni S, Ippolito A, Illei GG (2011) Helicobacter pylori and autoimmune diseases. Oral Dis 17:621–627
Schwimmer JB, Behling C, Newbury R, Deutsch R, Nievergelt C, Schork NJ, Lavine JE (2005) Histopathology of pediatric nonalcoholic fatty liver disease. Hepatology 42:641–649
Jian J, Li G, Hettinghouse A, Liu C (2016) Progranulin: a key player in autoimmune diseases. Cytokine. doi:10.1016/j.cyto.2016.08.007
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This paper has been edited and reviewed by medical editors from the Department of International Medical Communications of Tokyo Medical University.
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Kashiwagi, Y., Hatsushika, T., Tsutsumi, N. et al. Gastrointestinal and liver lesions in primary childhood Sjögren syndrome. Clin Rheumatol 36, 1433–1435 (2017). https://doi.org/10.1007/s10067-017-3599-4
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DOI: https://doi.org/10.1007/s10067-017-3599-4