KEGG   DISEASE: Sjogren syndrome
H01502                      Disease                                
Sjogren syndrome
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.
Immune system disease
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A43  Overlap or undifferentiated nonorgan specific systemic autoimmune disease
    H01502  Sjogren syndrome
hsa04658  Th1 and Th2 cell differentiation
hsa04659  Th17 cell differentiation
hsa04612  Antigen processing and presentation
hsa04630  JAK-STAT signaling pathway
hsa04620  Toll-like receptor signaling pathway
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
IRF5 (polymorphism) [HSA:3663] [KO:K09446]
STAT4 (polymorphism) [HSA:6775] [KO:K11222]
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
Stiller M, Golder W, Doring E, Biedermann T
Primary and secondary Sjogren's syndrome in children--a comparative study.
Clin Oral Investig 4:176-82 (2000)
van Nimwegen JF, Moerman RV, Sillevis Smitt N, Brouwer E, Bootsma H, Vissink A
Safety of treatments for primary Sjogren's syndrome.
Expert Opin Drug Saf 15:513-24 (2016)
Low HZ, Witte T
Aspects of innate immunity in Sjogren's syndrome.
Arthritis Res Ther 13:218 (2011)
Javaid MA, Ahmed AS, Durand R, Tran SD
Saliva as a diagnostic tool for oral and systemic diseases.
J Oral Biol Craniofac Res 6:66-75 (2016)
Baldini C, Giusti L, Ciregia F, Da Valle Y, Giacomelli C, Donadio E, Sernissi F, Bazzichi L, Giannaccini G, Bombardieri S, Lucacchini A
Proteomic analysis of saliva: a unique tool to distinguish primary Sjogren's syndrome from secondary Sjogren's syndrome and other sicca syndromes.
Arthritis Res Ther 13:R194 (2011)
Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindall E, Suen JY
Cevimeline for the treatment of xerostomia in patients with Sjogren syndrome: a randomized trial.
Arch Intern Med 162:1293-300 (2002)
Vitali C, Palombi G, Cataleta P
Treating Sjogren's Syndrome: Insights for the Clinician.
Ther Adv Musculoskelet Dis 2:155-66 (2010)

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