KEGG   DISEASE: シェーグレン症候群
エントリ  
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
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  非器官特異的全身性自己免疫疾患
   4A43  重複または未分化の非器官特異的全身性自己免疫疾患
    H01502  シェーグレン症候群
指定難病 [jp08407.html]
 H01502
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]
リンク   
ICD-11: 4A43.2
ICD-10: M35.0
MeSH: D012859
OMIM: 270150
文献    
  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
文献    
  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
文献    
  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
文献    
  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
文献    
  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
文献    
  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
文献    
  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

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