MCID: PRM294
MIFTS: 37

Primary Sjögren Syndrome

Categories: Bone diseases, Rare diseases, Immune diseases

Aliases & Classifications for Primary Sjögren Syndrome

MalaCards integrated aliases for Primary Sjögren Syndrome:

Name: Primary Sjögren Syndrome 59
Primary Sjögren-Gougerot Syndrome 59
Sjogrens Syndrome Primary 55

Classifications:



External Ids:

Orphanet 59 ORPHA289390

Summaries for Primary Sjögren Syndrome

MalaCards based summary : Primary Sjögren Syndrome, also known as primary sjögren-gougerot syndrome, is related to neonatal lupus erythematosus and transient neonatal thrombocytopenia. An important gene associated with Primary Sjögren Syndrome is TROVE2 (TROVE Domain Family Member 2), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Miconazole and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include eye, b cells and bone, and related phenotypes are Decreased viability and Decreased viability

Related Diseases for Primary Sjögren Syndrome

Diseases in the Sjogren Syndrome family:

Sjogren's Syndrome, Juvenile, Secondary to Autoimmune Disease Primary Sjögren Syndrome

Diseases related to Primary Sjögren Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 150)
# Related Disease Score Top Affiliating Genes
1 neonatal lupus erythematosus 10.9 SPTAN1 TRIM21
2 transient neonatal thrombocytopenia 10.9 CD79A TRIM21
3 hyperlucent lung 10.8 IL10 IL2RA
4 transient neonatal neutropenia 10.8 CD79A TRIM21
5 third-degree atrioventricular block 10.8 TRIM21 TROVE2
6 ross river fever 10.7 CD40LG IL10
7 immunodeficiency with hyper-igm, type 4 10.7 CD40LG TNFSF13B
8 selective igg deficiency disease 10.7 CD40LG CD79A
9 c1q nephropathy 10.7 CD40LG CD79A
10 meningovascular neurosyphilis 10.7 CD40LG CD79A
11 alpha chain disease 10.7 CD40LG CD79A
12 brill-zinsser disease 10.7 CD40LG CD79A
13 exudative glomerulonephritis 10.7 CD40LG CD79A
14 cork-handlers' disease 10.7 CD40LG CD79A
15 xerophthalmia 10.7 HSPG2 TRIM21
16 salpingo-oophoritis 10.7 CD40LG CD79A
17 early yaws 10.6 CD40LG CD79A
18 ventilation pneumonitis 10.6 CD40LG CD79A
19 chronic interstitial cystitis 10.6 CD40LG CD79A
20 axillary adenitis 10.6 CD40LG CD79A
21 cryofibrinogenemia 10.6 CD40LG CD79A
22 necrotizing ulcerative gingivitis 10.6 CD40LG IL10
23 trichostrongyloidiasis 10.6 CD40LG CD79A
24 subacute bacterial endocarditis 10.6 CD40LG CD79A
25 heterophyiasis 10.6 CD40LG CD79A
26 immunoglobulin g deficiency 10.6 CD40LG CD79A
27 orbital granuloma 10.6 CD40LG CD79A
28 cow milk allergy 10.6 CD79A IL10
29 transient hypogammaglobulinemia of infancy 10.6 CD40LG CD79A
30 gastroduodenitis 10.6 CD40LG CD79A
31 bacterial conjunctivitis 10.6 CD40LG CD79A
32 heavy chain disease 10.6 CD40LG CD79A
33 ophthalmia neonatorum 10.6 CD40LG CD79A
34 parotid disease 10.6 CD40LG CD79A
35 cerebral arteritis 10.6 CD40LG CD79A
36 selective immunoglobulin deficiency disease 10.6 CD40LG CD79A
37 sialadenitis 10.6 HSPG2 TNFSF13B TRIM21
38 geniculate herpes zoster 10.6 CD40LG CD79A
39 erythema elevatum diutinum 10.6 CD40LG CD79A
40 fallopian tube disease 10.6 CD40LG CD79A
41 sclerosing keratitis 10.6 B2M CD79A
42 hyperimmunoglobulin syndrome 10.5 CD40LG CD79A
43 congenital syphilis 10.5 CD40LG CD79A
44 lacrimal apparatus disease 10.5 HSPG2 IL10 TRIM21
45 pouchitis 10.5 IL10 LTF
46 polyclonal hypergammaglobulinemia 10.5 CD40LG CD79A
47 extrinsic allergic alveolitis 10.5 CD79A IL10 IL2RA
48 monoclonal paraproteinemia 10.5 CD40LG CD79A
49 salpingitis 10.5 CD40LG CD79A
50 ocular toxoplasmosis 10.5 CD40LG CD79A

Graphical network of the top 20 diseases related to Primary Sjögren Syndrome:



Diseases related to Primary Sjögren Syndrome

Symptoms & Phenotypes for Primary Sjögren Syndrome

GenomeRNAi Phenotypes related to Primary Sjögren Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00381-A-1 9.28 CD79A CHRM3 LTF
2 Decreased viability GR00381-A-3 9.28 CD79A CHRM3 LTF
3 Decreased viability GR00402-S-2 9.28 CD79A CHRM3 LTF

MGI Mouse Phenotypes related to Primary Sjögren Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.14 TROVE2 B2M CD40LG CD79A FAS HSPG2
2 cellular MP:0005384 10.13 TROVE2 B2M CALR CD40LG CD79A FAS
3 homeostasis/metabolism MP:0005376 10.11 B2M CALR CD40LG CD79A CHRM3 FAS
4 cardiovascular system MP:0005385 10.06 B2M CALR CD40LG CHRM3 FAS HSPG2
5 immune system MP:0005387 10.06 B2M CD40LG CD79A FAS HSPG2 IL10
6 integument MP:0010771 9.8 B2M CD40LG FAS HSPG2 IL10 TRIM21
7 liver/biliary system MP:0005370 9.7 B2M CD79A CHRM3 FAS HSPG2 IL10
8 nervous system MP:0003631 9.61 B2M CALR CD40LG CD79A CHRM3 FAS
9 renal/urinary system MP:0005367 9.1 TROVE2 CD40LG CD79A CHRM3 FAS TRIM21

Drugs & Therapeutics for Primary Sjögren Syndrome

Drugs for Primary Sjögren Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 81)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 2 22916-47-8 4189
2
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
3
Calcium Carbonate Approved, Investigational Phase 4 471-34-1
4
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
5
Pilocarpine Approved, Investigational Phase 4 54-71-7, 92-13-7 5910
6
Castor oil Approved, Investigational, Nutraceutical, Vet_approved Phase 4 8001-79-4
7 Gallopamil Investigational Phase 4 16662-47-8
8 glucocorticoids Phase 4
9 Gastrointestinal Agents Phase 4
10 Cyclosporins Phase 4,Phase 2
11 Hormone Antagonists Phase 4
12 Dermatologic Agents Phase 4,Phase 2
13 Hormones Phase 4
14 Antacids Phase 4
15 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
16 Immunosuppressive Agents Phase 4,Phase 3,Phase 2
17 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
18 Antifungal Agents Phase 4,Phase 2
19 Anti-Inflammatory Agents Phase 4
20 Anti-Ulcer Agents Phase 4
21 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1
22 Calcineurin Inhibitors Phase 4,Phase 2
23 Calcium, Dietary Phase 4,Not Applicable
24 Antineoplastic Agents, Hormonal Phase 4
25 Cathartics Phase 4
26 Cholinergic Agents Phase 4
27 Tetrahydrozoline Phase 4,Not Applicable
28 Peripheral Nervous System Agents Phase 4,Phase 2
29 Laxatives Phase 4
30 Carbopol 940 Phase 4,Not Applicable
31 Neurotransmitter Agents Phase 4
32 Autonomic Agents Phase 4
33 Castor Nutraceutical Phase 4
34
Hydroxychloroquine Approved Phase 3 118-42-3 3652
35
Abatacept Approved Phase 3,Phase 2 332348-12-6 10237
36 Antiparasitic Agents Phase 3
37 Antiprotozoal Agents Phase 3
38 Antimalarials Phase 3
39
Menthol Approved Phase 2,Not Applicable 2216-51-5 16666
40
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
41
belimumab Approved Phase 2 356547-88-1 5957 10451420
42
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4
43
Dehydroepiandrosterone Approved, Investigational, Nutraceutical Phase 2 53-43-0 9860744
44 Interleukin-2 Phase 2
45 Analgesics Phase 2
46 Adjuvants, Immunologic Phase 2
47 Analgesics, Non-Narcotic Phase 2
48 Pharmaceutical Solutions Phase 2,Phase 1,Not Applicable
49 Antibodies Phase 2,Phase 1
50 Immunoglobulins Phase 2,Phase 1

Interventional clinical trials:

(show top 50) (show all 60)
# Name Status NCT ID Phase Drugs
1 Cyclosporine A in the TReatment of Interstitial Pneumonitis Associated With Sjogren's Syndrome Unknown status NCT02370550 Phase 4 Cyclosporin A;Prednisone;Placebo;Calcium carbonate D
2 Quality of Life and Sjögren Syndrome Completed NCT03578900 Phase 4 Xeros;Citric Acid based Mouthwash
3 Safety Study of the Use of Topical Cyclosporine in the Treatment of Dry Eye Disease Completed NCT02004067 Phase 4 Restasis;Refresh Endura
4 Effect of Pilocarpine in Patients With Xerostomia Recruiting NCT02982577 Phase 4 Pilocarpine
5 Evaluation of Influenza A/H3N2 Vaccine in Patients With Rheumatologic Diseases Recruiting NCT03540823 Phase 4
6 Efficacy of Tocilizumab in Primary Sjögren's Syndrome. Unknown status NCT01782235 Phase 2, Phase 3 Tocilizumab;Placebo
7 Hydroxychloroquine Versus Placebo in Primary Sjögren's Syndrome Completed NCT00632866 Phase 3 Hydroxychloroquine;Placebo
8 Hydroxychloroquine Treatment of Dry Eyes in Patients With Primary Sjögren's Syndrome Completed NCT01601028 Phase 3 Hydroxychloroquine;Placebo
9 Efficacy and Safety of Abatacept in Patients With Primary Sjögren's Syndrome Recruiting NCT02067910 Phase 3 Abatacept SC
10 A Study to Assess the Efficacy and Safety of Abatacept in Adults With Active Primary Sjögrens Syndrome Active, not recruiting NCT02915159 Phase 3
11 Acupuncture for Primary Sjögren Syndrome Unknown status NCT02691377 Phase 2
12 Allogeneic Mesenchymal Stem Cells Transplantation for Primary Sjögren's Syndrome (pSS) Unknown status NCT00953485 Phase 1, Phase 2
13 A Phase II Study With Low-dose Recombinant Human IL-2 for the Treatment of Primary Sjögren's Syndrome Completed NCT02464319 Phase 2 hrIL-2 active;hrIL-2 placebo
14 Low Dose Cyclosporin A in Primary Sjögren Syndrome Completed NCT01693393 Phase 2 Cyclosporine A
15 PD of VAY736 in Patients With Primary Sjögren's Syndrome Completed NCT02149420 Phase 2 VAY736;Placebo
16 Safety, Pharmacokinetics, and Preliminary Efficacy Study of CDZ173 in Patients With Primary Sjögren's Syndrome Completed NCT02775916 Phase 2 CDZ173;Placebo
17 Effect of B-cell Depletion in Patients With Primary Sjögren's Syndrome Completed NCT00426543 Phase 2 MabThera (rituximab);Rituximab, Mabthera
18 A Study to Assess the Efficacy of RO5459072 in Participants With Primary Sjogren's Syndrome Completed NCT02701985 Phase 2 Placebo;RO5459072
19 Fatigue and Interleukin-1 (IL-1) Blockade in Primary Sjøgrens Syndrome Completed NCT00683345 Phase 2 Anakinra;Placebo
20 Dehydroepiandrosterone Administration in Women With Systemic Lupus Erythematosus or Primary Sjögren's Syndrome Completed NCT00391924 Phase 2 Dehydroepiandrosterone
21 Efficacy and Safety of Belimumab in Primary Sjögren's Syndrome Completed NCT01008982 Phase 2 LimphoStat-B
22 Efficacy and Safety of Belimumab in Subjects With Primary Sjögren's Syndrome Completed NCT01160666 Phase 2 Belimumab
23 A Safety and Efficacy Study of 552-02 in Subjects With Dry Mouth Associated With Sjogren's Syndrome Completed NCT00852839 Phase 2 Placebo;552-02
24 Rituximab Treatment in Sjogren's Syndrome Completed NCT00363350 Phase 1, Phase 2 rituximab (anti-CD20)
25 Lacripep™ in Subjects With Dry Eye Associated With Primary Sjögren's Syndrome Recruiting NCT03226444 Phase 1, Phase 2 0.005% Lacripep;0.01% Lacripep;Placebo
26 Safety, Pharmacokinetics and Preliminary Efficacy Study of CFZ533 in Patients With Primary Sjögren's Syndrome Recruiting NCT02291029 Phase 2 CFZ533 active - Cohort 1;CFZ533 placebo- Cohort 1;CFZ533 active - Cohort 2;CFZ533 placebo - Cohort 2;CFZ533 active -Cohort 3;CFZ533 active - Cohort 3
27 Safety and Efficacy of VAY736 in Patients With Primary Sjogren's Syndrome (pSS) Recruiting NCT02962895 Phase 2
28 Safety and Efficacy Study of Subcutaneous Belimumab and Intravenous Rituximab Co-administration in Subjects With Primary Sjogren's Syndrome Recruiting NCT02631538 Phase 2 Belimumab;Rituximab;Placebo belimumab;Placebo rituximab
29 A Phase 2a, Randomized, Placebo Controlled, Study to Evaluate the Safety and Efficacy of AMG 557/MEDI5872 in Primary Sjögren's Syndrome Active, not recruiting NCT02334306 Phase 2
30 A Study of RSLV-132 in Subjects With Primary Sjogren's Syndrome Active, not recruiting NCT03247686 Phase 2 RSLV-132;Placebo
31 UCB Proof of Concept Study in Patients With Primary Sjögren's Syndrome Terminated NCT02610543 Phase 2 UCB5857;Placebo
32 Proof of Concept Study to Evaluate the Efficacy and Safety of BMS-931699 (Lulizumab) or BMS-986142 in Primary Sjögren's Syndrome Terminated NCT02843659 Phase 2 BMS-931699;BMS-986142;Placebo
33 Baminercept, a Lymphotoxin-Beta Receptor Fusion Protein, for Treatment of Sjögren's Syndrome Terminated NCT01552681 Phase 2
34 Study of Pharmacodynamic Effects of VAY736 in Patients With Primary Sjögren's Syndrome Withdrawn NCT02495129 Phase 1, Phase 2 VAY736 lower dose;VAY736 higher dose
35 A Study to Evaluate the Safety, Tolerability, Pharmacokinetics (PK), Proof of Mechanism of GSK2618960 in Primary Sjögren's Syndrome (pSS) Withdrawn NCT03239600 Phase 2 GSK2618960 2 mg/kg;Placebo;Methotrexate
36 Abatacept for Patients With Inflammatory Arthritis Associated With Sjögren's Syndrome: an Open-Label Phase II Study Withdrawn NCT02027298 Phase 2 Abatacept
37 Mycophenolate Sodium Treatment in Patients With Primary Sjogren's Syndrome Completed NCT00542763 Phase 1 Mycophenolate sodium
38 A PH I Pilot Imaging Study to Evaluate Molecular Imaging Methods in HVs and pSS Pts Completed NCT02899377 Phase 1
39 Anti-CD20 Antibody Therapy for Sjogren's Syndrome Completed NCT00101829 Phase 1 Rituximab
40 Primary Sjögren Syndrome Unknown status NCT01989819
41 the Effect of HCG Treatment as a Cardiovascular Disease Morbidity Factor in Sjogren Syndrome Patients Unknown status NCT02381587
42 Confocal Microscopy and Lacrimal Gland in Sjogren's Syndrome Unknown status NCT00920179
43 Exercise in Sjogren, Myositis and Takayasu's Arteritis Unknown status NCT01501019 Not Applicable
44 Neurotrophins Implications in Primary Sjögren Syndrome Completed NCT01081184
45 Resistance Exercise in Woman With Primary Sjörgen Syndrome. Completed NCT03130062 Not Applicable
46 A Longitudinal, Observational Biomarker Study in Participants With Primary Sjogren's Syndrome Completed NCT02422407
47 Laser Therapy to Treat the Dry Mouth of Sjogren's Syndrome Completed NCT02066896 Not Applicable
48 Aerobic Exercise in Primary Sjögren's Syndrome Completed NCT02370225 Not Applicable
49 Evaluation of the Role of the Autonomic Nervous System in Sj(SqrRoot)(Delta)Gren s Syndrome Completed NCT00565526
50 The Functioning of Immune and Hormonal Systems in Patients With Sjogren's Syndrome and in Healthy Volunteers Completed NCT00001953

Search NIH Clinical Center for Primary Sjögren Syndrome

Genetic Tests for Primary Sjögren Syndrome

Anatomical Context for Primary Sjögren Syndrome

MalaCards organs/tissues related to Primary Sjögren Syndrome:

41
Eye, B Cells, Bone

Publications for Primary Sjögren Syndrome

Variations for Primary Sjögren Syndrome

Expression for Primary Sjögren Syndrome

Search GEO for disease gene expression data for Primary Sjögren Syndrome.

Pathways for Primary Sjögren Syndrome

Pathways related to Primary Sjögren Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.61 B2M CALR CD40LG CD79A IL10 IL2RA
2
Show member pathways
12.81 B2M CD40LG IL10 IL2RA SPTAN1 TNFSF13B
3
Show member pathways
12.59 CD40LG CD79A FAS TNFSF13B TRB
4
Show member pathways
12.3 B2M CALR CD40LG FAS IL10 IL2RA
5
Show member pathways
11.97 CD40LG FAS IL10 TRB
6 11.68 B2M CD40LG CD79A FAS IL10 IL2RA
7 11.64 CD79A IL10 IL2RA
8
Show member pathways
11.6 B2M IL2RA TRB

GO Terms for Primary Sjögren Syndrome

Cellular components related to Primary Sjögren Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.91 B2M CALR CD40LG HSPG2 IL10 LTF
2 extracellular region GO:0005576 9.81 B2M CALR CD40LG FAS HSPG2 IL10
3 specific granule lumen GO:0035580 9.5 B2M LTF SPTAN1
4 tertiary granule lumen GO:1904724 9.43 B2M LTF SPTAN1
5 cell surface GO:0009986 9.43 B2M CALR CD40LG FAS IL2RA LTF
6 external side of plasma membrane GO:0009897 9.02 B2M CALR CD40LG CD79A IL2RA

Biological processes related to Primary Sjögren Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.83 B2M CD79A IL2RA LTF TNFSF13B
2 inflammatory response GO:0006954 9.72 CD40LG FAS HSPG2 IL10 IL2RA
3 tumor necrosis factor-mediated signaling pathway GO:0033209 9.65 CD40LG FAS TNFSF13B
4 B cell differentiation GO:0030183 9.58 CD40LG CD79A IL10
5 positive regulation of T cell proliferation GO:0042102 9.54 CD40LG IL2RA TNFSF13B
6 response to molecule of bacterial origin GO:0002237 9.46 B2M IL10
7 positive regulation of cell cycle GO:0045787 9.33 CALR IL10 TRIM21
8 immunoglobulin secretion GO:0048305 9.26 CD40LG TNFSF13B
9 B cell proliferation GO:0042100 9.13 CD40LG CD79A IL10
10 immune response GO:0006955 9.1 B2M CD40LG FAS IL10 IL2RA TNFSF13B

Sources for Primary Sjögren Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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