SJOGREN SYNDROME

General Information (adopted from Orphanet):

Synonyms, Signs: SICCA SYNDROME
Number of Symptoms 7
OrphanetNr:
OMIM Id: 270150
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal recessive inheritance
[Omim]
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: No data available.

Symptom Information: Sort by abundance 

1
(HPO:0000217) Xerostomia 35 / 7739
2
(HPO:0001097) Keratoconjunctivitis sicca 25 / 7739
3
(HPO:0001370) Rheumatoid arthritis 12 / 7739
4
(HPO:0001939) Abnormality of metabolism/homeostasis 328 / 7739
5
(HPO:0002960) Autoimmunity 78 / 7739
6
(HPO:0000007) Autosomal recessive inheritance 2538 / 7739
7
(OMIM) Strong association with HLA-Dw3 and HLA-Dw4 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006).

See 200400 for association of Sjogren syndrome with achalasia in sisters. ...

Clinical Description OMIM Lichtenfeld et al. (1976) noted familial occurrence. This probably represents the same sort of familial occurrence as is seen with systemic lupus erythematosus (SLE; 152700) and other autoimmune disorders. Moutsopoulos et al. (1979) used the term primary or ...
Molecular genetics OMIM Gottenberg et al. (2003) compared 149 patients fulfilling the American-European Consensus Group criteria for Sjogren syndrome to 222 controls and confirmed the association of Sjogren syndrome with HLA alleles DRB1*03 (see HLA-DRB1; 142857) and DQB1*02 (see HLA-DQB1; 604305). ...