KEGG   DISEASE: Sjogren syndromeHelp
Entry
H01502                      Disease                                

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; Skin and connective tissue disease
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H01502  Sjogren syndrome
 Skin diseases
  Skin and soft tissue diseases
   H01502  Sjogren syndrome
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Non-organ specific systemic autoimmune disorders
   4A43  Overlap or undifferentiated non-organ specific systemic autoimmune disease
    H01502  Sjogren syndrome
BRITE hierarchy
Pathway
hsa04620 Toll-like receptor signaling pathway   
hsa04630 Jak-STAT signaling pathway   
Gene
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
IRF5 (polymorphism) [HSA:3663] [KO:K09446]
STAT4 (polymorphism) [HSA:6775] [KO:K11222]
Drug
Prednisolone sodium phosphate [DR:D00981]
Prednisone [DR:D00473]
Pilocarpine hydrochloride [DR:D02200]
Cevimeline hydrochloride [DR:D00661]
Other DBs
ICD-11: 4A43.2
ICD-10: M35.0
MeSH: D012859
OMIM: 270150
Reference
  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
Reference
  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
Reference
  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
Reference
PMID:26937373 (marker)
  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
Reference
PMID:22117835 (marker)
  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
Reference
PMID:12038948 (drug)
  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
Reference
PMID:22870445 (drug)
  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
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