| H01502 | |
| H number | H01502 |
| Name | Sjogren syndrome |
| Description | Sjogren Syndrome (SS) is a chronic inflammatory systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to sicca symptoms of the eyes and mouth. Several systemic and extraglandular manifestations can develop, including fatigue, arthritis, and involvement of organs such as the skin, lungs, and kidneys. SS may occur as a primary disorder or in association with other systemic autoimmune diseases, traditionally defined as secondary SS, such as rheumatoid arthritis, and systemic lupus erythematosus. Salivary secretions from these patients exhibit elevated levels of antibodies and cytokines. This is accompanied by a reduction in oral phosphate levels and xerostomia due to reduced salivary flow, which can lead to infections, progressive caries, dysphagia and oral pain. Current tests for SS include sialometry, salivary scintigraphy, sialography, serological tests or minor salivary gland biopsies. Recently, salivary biomarkers of SS has been investigated. The etiology of SS is still unclear. Since there is a familial aggregation of primary SS, however, genetic factors have been suspected for a long time. Initially, HLA haplotypes were shown to be associated with primary SS. Recently, polymorphisms in the genes IRF5 and STAT4 have been convincingly identified and replicated in several studies as susceptibility factors. |
| Category | Immune system disease |
| Network | - |
| Gene | HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752] IRF5 (polymorphism) [HSA:3663] [KO:K09446] STAT4 (polymorphism) [HSA:6775] [KO:K11222] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Prednisolone sodium phosphate [DR:D00981] Prednisone [DR:D00473] Pilocarpine hydrochloride [DR:D02200] Cevimeline hydrochloride [DR:D00661] |
| Comment | - |
| Other DBs | ICD-11: 4A43.2 ICD-10: M35.0 MeSH: D012859 OMIM: 270150 |
| Reference | PMID:11000325 AUTHORS Stiller M, Golder W, Doring E, Biedermann T TITLE Primary and secondary Sjogren's syndrome in children--a comparative study. JOURNAL Clin Oral Investig 4:176-82 (2000) DOI:10.1007/s007840000040176.784 PMID:26809028 AUTHORS van Nimwegen JF, Moerman RV, Sillevis Smitt N, Brouwer E, Bootsma H, Vissink A TITLE Safety of treatments for primary Sjogren's syndrome. JOURNAL Expert Opin Drug Saf 15:513-24 (2016) DOI:10.1517/14740338.2016.1146676 PMID:21635716 AUTHORS Low HZ, Witte T TITLE Aspects of innate immunity in Sjogren's syndrome. JOURNAL Arthritis Res Ther 13:218 (2011) DOI:10.1186/ar3318 PMID:26937373 AUTHORS Javaid MA, Ahmed AS, Durand R, Tran SD TITLE Saliva as a diagnostic tool for oral and systemic diseases. JOURNAL J Oral Biol Craniofac Res 6:66-75 (2016) DOI:10.1016/j.jobcr.2015.08.006 PMID:22117835 AUTHORS Baldini C, Giusti L, Ciregia F, Da Valle Y, Giacomelli C, Donadio E, Sernissi F, Bazzichi L, Giannaccini G, Bombardieri S, Lucacchini A TITLE Proteomic analysis of saliva: a unique tool to distinguish primary Sjogren's syndrome from secondary Sjogren's syndrome and other sicca syndromes. JOURNAL Arthritis Res Ther 13:R194 (2011) DOI:10.1186/ar3523 PMID:12038948 AUTHORS Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindall E, Suen JY TITLE Cevimeline for the treatment of xerostomia in patients with Sjogren syndrome: a randomized trial. JOURNAL Arch Intern Med 162:1293-300 (2002) DOI:10.1001/archinte.162.11.1293 PMID:22870445 AUTHORS Vitali C, Palombi G, Cataleta P TITLE Treating Sjogren's Syndrome: Insights for the Clinician. JOURNAL Ther Adv Musculoskelet Dis 2:155-66 (2010) DOI:10.1177/1759720X10363246 |