U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Links from PMC

Sjogren syndrome

MedGen UID:
282890
•Concept ID:
C1527336
•
Disease or Syndrome
Synonyms: SICCA SYNDROME; Sjogren's Syndrome; Sjögren's syndrome
SNOMED CT: Sjögren's disease (83901003); Sjögren's syndrome (83901003); Sjogren's syndrome (83901003); Sjögren syndrome (83901003); Sjogrens syndrome (83901003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0010030
OMIM®: 270150
Orphanet: ORPHA289390

Definition

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. [from OMIM]

Clinical features

From HPO
Interstitial nephritis
MedGen UID:
11952
•Concept ID:
C0041349
•
Disease or Syndrome
A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules.
See: Feature record | Search on this feature
Rheumatoid arthritis
MedGen UID:
2078
•Concept ID:
C0003873
•
Disease or Syndrome
Rheumatoid arthritis is an inflammatory disease, primarily of the joints, with autoimmune features and a complex genetic component.
See: Feature record | Search on this feature
Autoimmunity
MedGen UID:
2136
•Concept ID:
C0004368
•
Pathologic Function
The occurrence of an immune reaction against the organism's own cells or tissues.
See: Feature record | Search on this feature
Keratoconjunctivitis sicca
MedGen UID:
9620
•Concept ID:
C0022575
•
Disease or Syndrome
Dryness of the eye related to deficiency of the tear film components (aqueous, mucin, or lipid), lid surface abnormalities, or epithelial abnormalities. Keratoconjunctivitis sicca often results in a scratchy or sandy sensation (foreign body sensation) in the eyes, and may also be associated with itching, inability to produce tears, photosensitivity, redness, pain, and difficulty in moving the eyelids.
See: Feature record | Search on this feature
Abnormality of metabolism/homeostasis
MedGen UID:
867398
•Concept ID:
C4021768
•
Finding
See: Feature record | Search on this feature
Xerostomia
MedGen UID:
22735
•Concept ID:
C0043352
•
Disease or Syndrome
Dryness of the mouth due to salivary gland dysfunction.
See: Feature record | Search on this feature
Show allHide all

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSjogren syndrome

Professional guidelines

PubMed

Update on the pathophysiology and treatment of primary Sjögren syndrome.
Baldini C, Fulvio G, La Rocca G, Ferro F
Nat Rev Rheumatol 2024 Aug;20(8):473-491. Epub 2024 Jul 9 doi: 10.1038/s41584-024-01135-3. PMID: 38982205
EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.
Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Retamozo S, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vitali C, Bowman S, Mariette X; EULAR-Sjögren Syndrome Task Force Group
Ann Rheum Dis 2020 Jan;79(1):3-18. Epub 2019 Oct 31 doi: 10.1136/annrheumdis-2019-216114. PMID: 31672775
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article

Recent clinical studies

Etiology

Cardiovascular Involvement in Sjögren's Syndrome.
Atzeni F, Gozza F, Cafaro G, Perricone C, Bartoloni E
Front Immunol 2022;13:879516. Epub 2022 May 6 doi: 10.3389/fimmu.2022.879516. PMID: 35634284Free PMC Article
Autonomic neuropathies.
Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436
Osteoporosis in Rheumatic Diseases.
Adami G, Fassio A, Rossini M, Caimmi C, Giollo A, Orsolini G, Viapiana O, Gatti D
Int J Mol Sci 2019 Nov 22;20(23) doi: 10.3390/ijms20235867. PMID: 31766755Free PMC Article
Genetics in Sjögren Syndrome.
Reksten TR, Lessard CJ, Sivils KL
Rheum Dis Clin North Am 2016 Aug;42(3):435-47. Epub 2016 Jun 21 doi: 10.1016/j.rdc.2016.03.003. PMID: 27431346
Sjögren syndrome.
Mavragani CP, Moutsopoulos HM
CMAJ 2014 Oct 21;186(15):E579-86. Epub 2014 Feb 24 doi: 10.1503/cmaj.122037. PMID: 24566651Free PMC Article

Diagnosis

Sjögren syndrome.
Brito-Zerón P, Retamozo S, Ramos-Casals M
Med Clin (Barc) 2023 Feb 24;160(4):163-171. Epub 2022 Dec 15 doi: 10.1016/j.medcli.2022.10.007. PMID: 36528400
Primary Sjögren's syndrome and the eye.
Bjordal O, Norheim KB, Rødahl E, Jonsson R, Omdal R
Surv Ophthalmol 2020 Mar-Apr;65(2):119-132. Epub 2019 Oct 18 doi: 10.1016/j.survophthal.2019.10.004. PMID: 31634487
Sensory neuronopathies.
Gwathmey KG
Muscle Nerve 2016 Jan;53(1):8-19. Epub 2015 Nov 23 doi: 10.1002/mus.24943. PMID: 26467754
Sjögren syndrome.
Mavragani CP, Moutsopoulos HM
CMAJ 2014 Oct 21;186(15):E579-86. Epub 2014 Feb 24 doi: 10.1503/cmaj.122037. PMID: 24566651Free PMC Article
Primary Sjogren syndrome.
Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X
BMJ 2012 Jun 14;344:e3821. doi: 10.1136/bmj.e3821. PMID: 22700787

Therapy

Autonomic neuropathies.
Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436
EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.
Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Retamozo S, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vitali C, Bowman S, Mariette X; EULAR-Sjögren Syndrome Task Force Group
Ann Rheum Dis 2020 Jan;79(1):3-18. Epub 2019 Oct 31 doi: 10.1136/annrheumdis-2019-216114. PMID: 31672775
Lymphocytic Interstitial Pneumonia.
Panchabhai TS, Farver C, Highland KB
Clin Chest Med 2016 Sep;37(3):463-74. Epub 2016 Jun 25 doi: 10.1016/j.ccm.2016.04.009. PMID: 27514593
Sjögren Syndrome in the Twenty-First Century.
Scofield RH
Rheum Dis Clin North Am 2016 Aug;42(3):xiii-xiv. doi: 10.1016/j.rdc.2016.06.001. PMID: 27431355
Hydroxycholoroquine-induced hyperpigmentation.
Mir A, Boyd KP, Meehan SA, McLellan B
Dermatol Online J 2013 Dec 16;19(12):20723. PMID: 24365014

Prognosis

Endothelial Progenitor Cells in Autoimmune Disorders.
Feugray G, Miranda S, Le Cam Duchez V, Bellien J, Billoir P
Stem Cell Rev Rep 2023 Nov;19(8):2597-2611. Epub 2023 Sep 7 doi: 10.1007/s12015-023-10617-y. PMID: 37676423
Sjögren syndrome.
Brito-Zerón P, Retamozo S, Ramos-Casals M
Med Clin (Barc) 2023 Feb 24;160(4):163-171. Epub 2022 Dec 15 doi: 10.1016/j.medcli.2022.10.007. PMID: 36528400
Unique Aspects of Pediatric Sjögren Disease.
Randell RL, Lieberman SM
Rheum Dis Clin North Am 2021 Nov;47(4):707-723. Epub 2021 Aug 27 doi: 10.1016/j.rdc.2021.07.008. PMID: 34635300Free PMC Article
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article
Keratoconjunctivitis sicca.
Roberts DK
J Am Optom Assoc 1991 Mar;62(3):187-99. PMID: 1813522

Clinical prediction guides

Artificial intelligence-driven drug development against autoimmune diseases.
Moingeon P
Trends Pharmacol Sci 2023 Jul;44(7):411-424. Epub 2023 May 31 doi: 10.1016/j.tips.2023.04.005. PMID: 37268540
Cardiovascular Disease in Primary Sjögren's Syndrome.
Berardicurti O, Ruscitti P, Cipriani P, Ciccia F, Liakouli V, Guggino G, Carubbi F, Di Benedetto P, Triolo G, Giacomelli R
Rev Recent Clin Trials 2018;13(3):164-169. doi: 10.2174/1574887113666180315130336. PMID: 29542415
B cells in the pathogenesis of primary Sjögren syndrome.
Nocturne G, Mariette X
Nat Rev Rheumatol 2018 Mar;14(3):133-145. Epub 2018 Feb 8 doi: 10.1038/nrrheum.2018.1. PMID: 29416129
Polyautoimmunity in Sjögren Syndrome.
Anaya JM, Rojas-Villarraga A, Mantilla RD, Arcos-Burgos M, Sarmiento-Monroy JC
Rheum Dis Clin North Am 2016 Aug;42(3):457-72. Epub 2016 Jun 21 doi: 10.1016/j.rdc.2016.03.005. PMID: 27431348
Sjögren syndrome.
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M
Nat Rev Dis Primers 2016 Jul 7;2:16047. doi: 10.1038/nrdp.2016.47. PMID: 27383445

Recent systematic reviews

Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Sjögren Syndrome in Primary Salivary Gland Lymphoma.
Travaglino A, Giordano C, Pace M, Varricchio S, Picardi M, Pane F, Staibano S, Mascolo M
Am J Clin Pathol 2020 May 5;153(6):719-724. doi: 10.1093/ajcp/aqaa005. PMID: 32076706
Efficacy and safety of topical and systemic medications: a systematic literature review informing the EULAR recommendations for the management of Sjögren's syndrome.
Brito-Zerón P, Retamozo S, Kostov B, Baldini C, Bootsma H, De Vita S, Dörner T, Gottenberg JE, Kruize AA, Mandl T, Ng WF, Seror R, Tzioufas AG, Vitali C, Bowman S, Mariette X, Ramos-Casals M
RMD Open 2019;5(2):e001064. Epub 2019 Oct 28 doi: 10.1136/rmdopen-2019-001064. PMID: 31749986Free PMC Article
Systemic lupus erythematosus: An update for ophthalmologists.
Papagiannuli E, Rhodes B, Wallace GR, Gordon C, Murray PI, Denniston AK
Surv Ophthalmol 2016 Jan-Feb;61(1):65-82. Epub 2015 Jul 18 doi: 10.1016/j.survophthal.2015.06.003. PMID: 26197421
Acupuncture for treating dry eye: a systematic review.
Lee MS, Shin BC, Choi TY, Ernst E
Acta Ophthalmol 2011 Mar;89(2):101-6. doi: 10.1111/j.1755-3768.2009.01855.x. PMID: 20337604

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...