International Journal of Celiac Disease
ISSN (Print): 2334-3427 ISSN (Online): 2334-3486 Website: Editor-in-chief: Samasca Gabriel
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International Journal of Celiac Disease. 2020, 8(3), 76-82
DOI: 10.12691/ijcd-8-3-1
Open AccessArticle

Sjögren’s Syndrome Associated with Celiac Disease

Felipe Freire Silva1, , Aaron Lerner2, João Carlos Geber Júnior1, Jozélio Freire de Carvalho3 and Yehuda Shoenfeld2, 4

1Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

2Chaim Sheba Medical Center, the Zabludowicz Center, for Autoimmune Diseases Tel-Hashomer, Israel

3Institute for Health Sciences from Federal University of Bahia, Salvador, Bahia, Brazil

4I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

Pub. Date: August 02, 2020

Cite this paper:
Felipe Freire Silva, Aaron Lerner, João Carlos Geber Júnior, Jozélio Freire de Carvalho and Yehuda Shoenfeld. Sjögren’s Syndrome Associated with Celiac Disease. International Journal of Celiac Disease. 2020; 8(3):76-82. doi: 10.12691/ijcd-8-3-1


Background: Autoimmune diseases tend to run in families and the affected persons are prone to additional autoimmune conditions. This is the case for celiac disease (CD) and Sjögren's syndrome (SS). Objectives: To describe a patient with both diseases and to analyze the clinical, therapeutic and evolutionary characteristics of published patients with SS associated with CD. Methodology: A systematic review of articles published in PubMed, MEDLINE, LILACS and Scielo dating from 1966 to May 2020, was conducted, using the following search words: Sjögren's syndrome, Celiac disease. Only English, German and French publications were considered. Results: Only 16 studies with 31 patients with SS associated CD were depicted. Adding the present case would mount the total to 17 studies, describing 32 patients. There are 6 observational studies and 10 case reports. In relation to demographics, age varied from 10 to 73 years old, with a median of 45 years and the great majority were female (21/32 patients). SS preceded CD manifestations in 7 studies, CD was the first presentation in 6 articles and simultaneous diseases was observed in 4 reports. Time elapsed between the two diseases varied from 2 to 33 years. Autoantibodies related to SS showed that anti-Ro and anti-La were positive in 8/17, while CD-related antibodies were positive in 12/17 studies. Regarding therapy, the majority received a gluten-free diet as CD therapy (13/17 studies). Glucocorticoid was prescribed in 6/17 studies and hydroxychloroquine in 4/17. When described, all studies demonstrated improvement of CD manifestations and 7/17 improved SS symptoms. Conclusion: The present comprehensive review evaluated all published cases of SS and CD of the literature. In the majority, SS precedes CD and all patients had good responses to appropriate therapy. It is hoped that increased awareness of the combination of SS and CD will result in earlier diagnosis and therapy and improved outcome.

Sjögren's syndrome celiac disease dermatitis herpetiformis autoantibodies poly-autoimmunity multiple autoimmune syndrome

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[1]  Mariette X, Criswell LA. Primary Sjögren's Syndrome. N Engl J Med. 2018; 378: 931-939.
[2]  Maciel G, Crowson CS, Matteson EL, Cornec D. Prevalence of Primary Sjögren's Syndrome in a US Population-Based Cohort. Arthritis Care Res (Hoboken). 2017; 69: 1612-16.
[3]  Alani H, Henty JR, Thompson NL, Jury E, Ciurtin C. Systematic Review and Meta-Analysis of the Epidemiology of Polyautoimmunity in Sjögren's Syndrome (Secondary Sjögren's Syndrome) Focusing on Autoimmune Rheumatic Diseases. Scand J Rheumatol. 2018; 47: 141-54.
[4]  Harpreet S, Jain Deepak J, Kiran B. Multiple autoimmune syndrome with celiac disease. Reumatologia. 2016; 54: 326-329.
[5]  Caio G, De Giorgio R, Ursini F, Fanaro S, Volta U. Prevalence of celiac disease serological markers in a cohort of Italian rheumatological patients. Gastroenterol Hepatol Bed Bench. 2018; 11: 244-249.
[6]  Lerner A, Jeremias P, Matthias T. The world incidence of celiac disease is increasing: a review. Internat. J. Of Recent Scient. Res. 2015; 7: 5491-5496.
[7]  Lerner A, Matthias T. A Silent or Hypo-symptomatic Disease Can Erupt: Acute Presentations of Celiac Disease. Internat J Celiac Did 2017; 5: 129-132.
[8]  Lerner A, Lopez F, Schmiedl A, Matthias T. The Underdiagnosed Enemy: Africa Goes Celiac? Internat. J Celiac Dis. 2019; 7: 9-12.
[9]  Lionetti E, Catassi C. Co-localization of gluten consumption and HLA-DQ2 and -DQ8 genotypes, a clue to the history of celiac disease. Dig Liver Dis. 2014; 46: 1057-63.
[10]  Tommasini A, Not T, Kiren V, Baldas V, Santon D, Trevisiol C, et al. Mass screening for celiac disease using antihuman transglutaminase antibody assay. Arch Dis Child 2004; 89: 512-515.
[11]  Lerner A. Matthias T. Autoimmunity in celiac disease: extra- intestinal manifestations. Autoimm. Rev. 2019; 18: 241-246.
[12]  Samasca G, Ramesh A, Sur D, Cornel A, Sur L, Floca E, SurG, Lupan L, Matthias T, Lerner A. Polyautoimmunity - The missing ingredient. Autoimmun Rev. 2018; 17: 840-841.
[13]  Mohan MP, Ramesh TC. Multiple autoimmune syndrome. Indian J Dermatol Venereol Leprol. 2003; 69: 298–299.
[14]  Erbasan F, Çoban DT, Karasu U, Çekin Y, Yesil B, Çekin AH, et al. Primary Sjögren's Syndrome in Patients With Celiac Disease. Turk J Med Sci. 2017; 47: 430-4.
[15]  Bibbò S, Pes GM, Usai-Satta P, Salis R, Soro S, Colosso BMQ, et al. Chronic Autoimmune Disorders Are Increased in Coeliac Disease: A Case-Control Study. 2017; 96: e8562.
[16]  Lidén M, Kristjánsson G, Valtýsdóttir S, Hällgren R. Gluten Sensitivity in Patients With Primary Sjögren’s Syndrome. Scand J Gastroenterol. 2007; 42: 962-7.
[17]  Szodoray P, Barta Z, Lakos G, Szakáll S, Zeher M. Coeliac Disease in Sjögren's Syndrome--A Study of 111 Hungarian Patients. 2004; 24: 278-82.
[18]  Iltanen S, Collin P, Korpela M, Holm K, Partanen J, Polvi A, et al. Celiac Disease and Markers of Celiac Disease Latency in Patients With Primary Sjögren's Syndrome. 1999; 94: 1042-6.
[19]  Khammassi N, Mohsen D, Kort Y, Abdelhedi H, Cherify O. Association Gougerot Sjogren Syndrome and Celiac Disease. Pan Afr Med J. 2015; 22: 33.
[20]  Nikiphorou E, Hall FC. First Report of Improvement of Coeliac Disease in a Patient With Sjögren's Syndrome Treated With Rituximab. Rheumatology (Oxford). 2014; 53: 1906-7.
[21]  Tinsa F, Brini I, El May A, Bousnina D, Boussetta K, Bousnina S. Nodular Regenerative Hyperplasia of the Liver, Celiac Disease and Sjogren's Syndrome in a Child. Gastroenterol Clin Biol. 2010; 34: 40-2.
[22]  Katsikas GA, Maragou M, Rontogianni D, Gouma P, Koutsouvelis I, Kappou-Rigatou I. Secondary Cutaneous Nodular AA Amyloidosis in a Patient With Primary Sjögren Syndrome and Celiac Disease. J Clin Rheumatol. 2008; 14: 27-9.
[23]  D’Onofrio F, Miele L, Diaco M, Santoro L, De Socio G, Montalto M, et al. Int J Immunopathol Pharmacol. 2006; 19: 445-8.
[24]  Fracchia M, Galatola G, Corradi F, Dall’Omo AM, Rovera L, Pera A, et al. Dig Liver Dis. 2004; 36: 489-91.
[25]  Kaufmann J, Schneider W, Schmidt W, Gromnica-Ihle E. Concomitant Dermatitis Herpetiformis Duhring, Arthritis and Sjögren Syndrome in a Patient With Celiac Disease. Z Rheumatol. 1998; 57: 114-7.
[26]  Whitehead EM, Daly JG, Hayes JR. Renal Tubular Acidosis in Association With Sjögren's Syndrome, Primary Biliary Cirrhosis and Coeliac Disease. Ir J Med Sci. 1987; 156: 124-5.
[27]  Maclaurin BP, Matthews N, Kilpatrick JA. Coeliac Disease Associated With Auto-Immune Thyroiditis, Sjogren's Syndrome, and a Lymphocytotoxic Serum Factor. Aust N Z J Med. 1972; 2: 405-11.
[28]  Pittman FE, Holub DA. Sjoegren's Syndrome and Adult Celiac Disease. 1965; 48: 869-76.
[29]  Lerner A, Shoenfeld Y, Matthias T. A Review: Gluten ingestion side effects and withdrawal advantages in non-celiac autoimmune diseases. 2017, Nutr Rev. 2017; 75: 1046-1058.
[30]  Lerner A, O’Bryan T, Matthias T. Navigating the gluten-free diet boom: the dark side of gluten free diet. Front Pediatr. 2019;7: Article 414.
[31]  Lerner A, Matthias T. Gluten free diet- tough ally in torrid time. Internat J of Celiac Disease 2017; 5: 50-55.
[32]  Lerner A, Matthias T. The Yin and Yang of dietary gluten transgressions in real-life scenarios of celiac patients. BMC Med. 2020; 18: 70-72
[33]  Colafrancesco S, Agmon-Levin N, Perricone C, Shoenfeld Y. Unraveling the soul of autoimmune diseases: pathogenesis, diagnosis and treatment adding dowels to the puzzle. Immunol Res. 2013; 56: 200-5.
[34]  Bartoloni E, Bistoni O, Alunno A, Cavagna L, Nalotto L, Baldini C, et al. Celiac Disease Prevalence Is Increased in Primary Sjögren’s Syndrome and Diffuse Systemic Sclerosis: Lessons from a Large Multi-Center Study. J Clin Med. 2019; 8: 540.
[35]  O'Neill CA, Monteleone G, McLaughlin JT, Paus R. The Gut-Skin Axis in Health and Disease: A Paradigm With Therapeutic Implications. Bioessays, 2016; 38: 1167-1176.
[36]  Lerner A, Matthias T. GUT-the Trojan horse in remote organs’ autoimmunity. Journal of Clinical & Cellular Immunology, 2016; 7: 401.
[37]  Lerner A, Aminov R, Matthias T. Dysbiosis may trigger autoimmune diseases via inappropriate posttranslational modification of host proteins. Front in Microbiol. 2016; 7: 84.
[38]  Lerner A, Aminov R, Matthias T. Potential effects of horizontal gene exchange in the human gut. Frontiers in Microbiol. 2017; 8: 1630.
[39]  Lerner A, Neidhöfer S, Matthias T. The gut microbiome feelings of the brain: perspective for Non-Microbiologists. Microorganisms, 2017; 5: 66.
[40]  Lerner A, Neidhöfer S, Matthias T. Transglutaminase 2 and anti transglutaminase 2 autoantibodies in celiac disease and beyond: Part A: TG2 double-edged sword: gut and extraintestinal involvement. Immunome Res. 2015; 11: 3.
[41]  Abenavoli L, Dastoli S, Bennardo L, Boccuto L, Passante M, Silvestri M, et al. The Skin in Celiac Disease Patients: The Other Side of the Coin. Medicina (Kaunas). 2019; 55: 578.
[42]  Lerner A, Matthias T. Rheumatoid arthritis–celiac disease relationship: Joints get that gut feeling. Autoimmunity Reviews 2015; 14: 1038–1047.
[43]  Lerner A, Jeremias P, Matthias T. The gut-thyroid axis and celiac disease. Endocrinol Connections, 2017; 6: R52-R58.
[44]  Lee SK, Kim YS, Lee YJ, Lee SS, Song IS, Park SC, et al. Transglutaminase 2 Expression in the Salivary Myoepithelial Cells of Mouse Embryo. Arch Oral Biol, 2005; 50: 301-8.
[45]  Hu S, Vissink A, Arellano M, et al. Identification of autoantibody biomarkers for primary Sjogren's syndrome using protein microarrays. Proteomics. 2011; 11: 1499-1507.
[46]  Lebwohl B, Sanders D, Green PHR. Coeliac Disease. Lancet. 2018; 391(10115): 70-81.