| H01502 | |
| H番号 | H01502 |
| 名称 | シェーグレン症候群 |
| 概要 | 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. |
| カテゴリ | 免疫系疾患 |
| ネットワーク | - |
| 病因遺伝子 | HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752] IRF5 (polymorphism) [HSA:3663] [KO:K09446] STAT4 (polymorphism) [HSA:6775] [KO:K11222] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | ピロカルピン塩酸塩 [DR:D02200] 精製ヒアルロン酸ナトリウム [DR:D03354] セビメリン塩酸塩水和物 [DR:D00661] リン酸二カリウム・塩化カリウム・塩化カルシウム水和物・塩化ナトリウム・塩化マグネシウム [DR:D04424] |
| コメント | - |
| リンク | ICD-11: 4A43.2 ICD-10: M35.0 MeSH: D012859 OMIM: 270150 |
| 文献 | PMID:11000325 著者 Stiller M, Golder W, Doring E, Biedermann T タイトル Primary and secondary Sjogren's syndrome in children--a comparative study. 雑誌 Clin Oral Investig 4:176-82 (2000) DOI:10.1007/s007840000040176.784 PMID:26809028 著者 van Nimwegen JF, Moerman RV, Sillevis Smitt N, Brouwer E, Bootsma H, Vissink A タイトル Safety of treatments for primary Sjogren's syndrome. 雑誌 Expert Opin Drug Saf 15:513-24 (2016) DOI:10.1517/14740338.2016.1146676 PMID:21635716 著者 Low HZ, Witte T タイトル Aspects of innate immunity in Sjogren's syndrome. 雑誌 Arthritis Res Ther 13:218 (2011) DOI:10.1186/ar3318 PMID:26937373 著者 Javaid MA, Ahmed AS, Durand R, Tran SD タイトル Saliva as a diagnostic tool for oral and systemic diseases. 雑誌 J Oral Biol Craniofac Res 6:66-75 (2016) DOI:10.1016/j.jobcr.2015.08.006 PMID:22117835 著者 Baldini C, Giusti L, Ciregia F, Da Valle Y, Giacomelli C, Donadio E, Sernissi F, Bazzichi L, Giannaccini G, Bombardieri S, Lucacchini A タイトル Proteomic analysis of saliva: a unique tool to distinguish primary Sjogren's syndrome from secondary Sjogren's syndrome and other sicca syndromes. 雑誌 Arthritis Res Ther 13:R194 (2011) DOI:10.1186/ar3523 PMID:12038948 著者 Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindall E, Suen JY タイトル Cevimeline for the treatment of xerostomia in patients with Sjogren syndrome: a randomized trial. 雑誌 Arch Intern Med 162:1293-300 (2002) DOI:10.1001/archinte.162.11.1293 PMID:22870445 著者 Vitali C, Palombi G, Cataleta P タイトル Treating Sjogren's Syndrome: Insights for the Clinician. 雑誌 Ther Adv Musculoskelet Dis 2:155-66 (2010) DOI:10.1177/1759720X10363246 |