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Sicca syndrome

MedGen UID:
39529
•Concept ID:
C0086981
•
Disease or Syndrome
Synonyms: Sicca Syndrome; Syndrome, Sicca
SNOMED CT: Sicca syndrome (83901003)
 
OMIM®: 270150

Definition

A constellation of symptoms that include abnormal dryness of the mouth, eyes and other mucous membranes. The condition is seen in patients with Sjogren syndrome, sarcoidosis, amyloidosis, and deficiencies of vitamins A and C. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

A Clinical Review of Primary Biliary Cholangitis.
Pullen R
Gastroenterol Nurs 2020 Mar/Apr;43(2):E48-E55. doi: 10.1097/SGA.0000000000000480. PMID: 32251226
Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment.
Both T, Dalm VA, van Hagen PM, van Daele PL
Int J Med Sci 2017;14(3):191-200. Epub 2017 Feb 23 doi: 10.7150/ijms.17718. PMID: 28367079Free PMC Article
Advances in the understanding and treatment of systemic complications in Sjögren's syndrome.
Brito-Zerón P, Ramos-Casals M; EULAR-SS task force group
Curr Opin Rheumatol 2014 Sep;26(5):520-7. doi: 10.1097/BOR.0000000000000096. PMID: 25050925

Recent clinical studies

Etiology

Sjögren's syndrome: shedding light on emerging and key drug targets.
Ridgewell D, Thalayasingam N, Ng WF
Expert Opin Ther Targets 2022 Oct;26(10):869-882. Epub 2022 Dec 28 doi: 10.1080/14728222.2022.2157259. PMID: 36576336
Labial gland-derived mesenchymal stem cells and their exosomes ameliorate murine Sjögren's syndrome by modulating the balance of Treg and Th17 cells.
Li B, Xing Y, Gan Y, He J, Hua H
Stem Cell Res Ther 2021 Aug 26;12(1):478. doi: 10.1186/s13287-021-02541-0. PMID: 34446113Free PMC Article
Sicca Syndrome Associated with Immune Checkpoint Inhibitor Therapy.
Warner BM, Baer AN, Lipson EJ, Allen C, Hinrichs C, Rajan A, Pelayo E, Beach M, Gulley JL, Madan RA, Feliciano J, Grisius M, Long L, Powers A, Kleiner DE, Cappelli L, Alevizos I
Oncologist 2019 Sep;24(9):1259-1269. Epub 2019 Apr 17 doi: 10.1634/theoncologist.2018-0823. PMID: 30996010Free PMC Article
Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment.
Both T, Dalm VA, van Hagen PM, van Daele PL
Int J Med Sci 2017;14(3):191-200. Epub 2017 Feb 23 doi: 10.7150/ijms.17718. PMID: 28367079Free PMC Article
Primary Sjögren's syndrome.
Rischmueller M, Tieu J, Lester S
Best Pract Res Clin Rheumatol 2016 Feb;30(1):189-220. Epub 2016 May 13 doi: 10.1016/j.berh.2016.04.003. PMID: 27421224

Diagnosis

Sjögren's syndrome: shedding light on emerging and key drug targets.
Ridgewell D, Thalayasingam N, Ng WF
Expert Opin Ther Targets 2022 Oct;26(10):869-882. Epub 2022 Dec 28 doi: 10.1080/14728222.2022.2157259. PMID: 36576336
TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review.
Grange L, Chalayer E, Boutboul D, Paul S, Galicier L, Gramont B, Killian M
Autoimmun Rev 2022 Aug;21(8):103137. Epub 2022 Jul 6 doi: 10.1016/j.autrev.2022.103137. PMID: 35803499
Pulmonary involvement in Sjögren's syndrome.
Mestre-Torres J, Solans-Laque R
Med Clin (Barc) 2022 Feb 25;158(4):181-185. Epub 2021 Aug 13 doi: 10.1016/j.medcli.2021.06.016. PMID: 34392987
Diagnosis and treatment of primary biliary cholangitis.
Laschtowitz A, de Veer RC, Van der Meer AJ, Schramm C
United European Gastroenterol J 2020 Jul;8(6):667-674. Epub 2020 Apr 16 doi: 10.1177/2050640620919585. PMID: 32299307Free PMC Article
Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment.
Both T, Dalm VA, van Hagen PM, van Daele PL
Int J Med Sci 2017;14(3):191-200. Epub 2017 Feb 23 doi: 10.7150/ijms.17718. PMID: 28367079Free PMC Article

Therapy

Sicca syndrome associated with immune checkpoint inhibitor therapy.
Harris JA, Huang K, Miloslavsky E, Hanna GJ
Oral Dis 2022 Nov;28(8):2083-2092. Epub 2021 Aug 17 doi: 10.1111/odi.14000. PMID: 34379884
Rheumatic immune-related adverse events induced by immune checkpoint inhibitors.
Zhong H, Zhou J, Xu D, Zeng X
Asia Pac J Clin Oncol 2021 Jun;17(3):178-185. Epub 2020 Jul 27 doi: 10.1111/ajco.13346. PMID: 32717098Free PMC Article
Diagnosis and treatment of primary biliary cholangitis.
Laschtowitz A, de Veer RC, Van der Meer AJ, Schramm C
United European Gastroenterol J 2020 Jul;8(6):667-674. Epub 2020 Apr 16 doi: 10.1177/2050640620919585. PMID: 32299307Free PMC Article
Distal renal tubular acidosis and severe hypokalemia: a case report and review of the literature.
Vasquez-Rios G, Westrich DJ Jr, Philip I, Edwards JC, Shieh S
J Med Case Rep 2019 Apr 26;13(1):103. doi: 10.1186/s13256-019-2056-1. PMID: 31023369Free PMC Article
Sicca Syndrome Associated with Immune Checkpoint Inhibitor Therapy.
Warner BM, Baer AN, Lipson EJ, Allen C, Hinrichs C, Rajan A, Pelayo E, Beach M, Gulley JL, Madan RA, Feliciano J, Grisius M, Long L, Powers A, Kleiner DE, Cappelli L, Alevizos I
Oncologist 2019 Sep;24(9):1259-1269. Epub 2019 Apr 17 doi: 10.1634/theoncologist.2018-0823. PMID: 30996010Free PMC Article

Prognosis

Not all autoantibodies are clinically relevant. Classic and novel autoantibodies in Sjögren's syndrome: A critical review.
Vílchez-Oya F, Balastegui Martin H, García-Martínez E, Corominas H
Front Immunol 2022;13:1003054. Epub 2022 Oct 17 doi: 10.3389/fimmu.2022.1003054. PMID: 36325321Free PMC Article
Pulmonary involvement in Sjögren's syndrome.
Mestre-Torres J, Solans-Laque R
Med Clin (Barc) 2022 Feb 25;158(4):181-185. Epub 2021 Aug 13 doi: 10.1016/j.medcli.2021.06.016. PMID: 34392987
Minor salivary gland biopsy: Its role in the classification and prognosis of Sjögren's syndrome.
Bautista-Vargas M, Vivas AJ, Tobón GJ
Autoimmun Rev 2020 Dec;19(12):102690. Epub 2020 Oct 22 doi: 10.1016/j.autrev.2020.102690. PMID: 33099041
Diagnosis and treatment of primary biliary cholangitis.
Laschtowitz A, de Veer RC, Van der Meer AJ, Schramm C
United European Gastroenterol J 2020 Jul;8(6):667-674. Epub 2020 Apr 16 doi: 10.1177/2050640620919585. PMID: 32299307Free PMC Article
Sjogren's syndrome is associated with and not secondary to systemic sclerosis.
Salliot C, Mouthon L, Ardizzone M, Sibilia J, Guillevin L, Gottenberg JE, Mariette X
Rheumatology (Oxford) 2007 Feb;46(2):321-6. Epub 2006 Jul 28 doi: 10.1093/rheumatology/kel252. PMID: 16877466

Clinical prediction guides

Not all autoantibodies are clinically relevant. Classic and novel autoantibodies in Sjögren's syndrome: A critical review.
Vílchez-Oya F, Balastegui Martin H, García-Martínez E, Corominas H
Front Immunol 2022;13:1003054. Epub 2022 Oct 17 doi: 10.3389/fimmu.2022.1003054. PMID: 36325321Free PMC Article
Maladaptive Autophagy in the Pathogenesis of Autoimmune Epithelitis in Sjögren's Syndrome.
Colafrancesco S, Barbati C, Priori R, Putro E, Giardina F, Gattamelata A, Monosi B, Colasanti T, Celia AI, Cerbelli B, Giordano C, Scarpa S, Fusconi M, Cavalli G, Berardicurti O, Gandolfo S, Nayar S, Barone F, Giacomelli R, De Vita S, Alessandri C, Conti F
Arthritis Rheumatol 2022 Apr;74(4):654-664. Epub 2022 Mar 1 doi: 10.1002/art.42018. PMID: 34748286
Minor salivary gland biopsy: Its role in the classification and prognosis of Sjögren's syndrome.
Bautista-Vargas M, Vivas AJ, Tobón GJ
Autoimmun Rev 2020 Dec;19(12):102690. Epub 2020 Oct 22 doi: 10.1016/j.autrev.2020.102690. PMID: 33099041
Clinical characteristics of rheumatic syndromes associated with checkpoint inhibitors therapy.
Kostine M, Truchetet ME, Schaeverbeke T
Rheumatology (Oxford) 2019 Dec 1;58(Suppl 7):vii68-vii74. doi: 10.1093/rheumatology/kez295. PMID: 31816082Free PMC Article
Clinical and epidemiological differences between men and women with systemic sclerosis: a study in a Spanish systemic sclerosis cohort and literature review.
Freire M, Rivera A, Sopeña B, Tolosa Vilella C, Guillén-Del Castillo A, Colunga Argüelles D, Callejas Rubio JL, Rubio Rivas M, Trapiella Martínez L, Todolí Parra JA, Rodríguez Carballeira M, Iniesta Arandia N, García Hernández FJ, Egurbide Arberas MV, Sáez Comet L, Vargas Hitos JA, Ríos Blanco JJ, Marín Ballvé A, Pla Salas X, Madroñero Vuelta AB, Ruiz Muñoz M, Fonollosa Pla V, Simeón Aznar CP; RESCLE investigators, Autoimmune Diseases Study Group (GEAS)
Clin Exp Rheumatol 2017 Sep-Oct;35 Suppl 106(4):89-97. Epub 2017 Sep 18 PMID: 28980905

Recent systematic reviews

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic).
Trevisani VFM, Pinheiro AC, de Magalhães Souza Fialho SC, Fernandes MLMS, Pugliesi A, Pasoto SG, Lopes MLL, Guedes LKN, Miyamoto ST, Dos Santos LC, Appenzeller S, Fidelix T, Ribeiro SLE, de Brito DCSE, Libório-Kimura TN, Santos MCLFS, Cantali DU, Gennari JD', Capobianco KG, Civile VT, Pinto ACPN, da Rocha AP, Rocha-Filho CR, Oliveira FR, Valim V
Adv Rheumatol 2022 Oct 10;62(1):35. doi: 10.1186/s42358-022-00267-y. PMID: 36217186
TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review.
Grange L, Chalayer E, Boutboul D, Paul S, Galicier L, Gramont B, Killian M
Autoimmun Rev 2022 Aug;21(8):103137. Epub 2022 Jul 6 doi: 10.1016/j.autrev.2022.103137. PMID: 35803499
Diagnostic performance of serology against histologic assessment to diagnose Sjogren's syndrome: a systematic review.
Viegas-Costa LC, Friesen R, Flores-Mir C, McGaw T
Clin Rheumatol 2021 Dec;40(12):4817-4828. Epub 2021 Jun 17 doi: 10.1007/s10067-021-05813-5. PMID: 34142295
Unstimulated whole salivary flow in Sjögren's Syndrome: systematic literature review and meta-analysis.
Martínez-Ceballos MA, Aguilera N, Garzón-González KC, Cajamarca-Baron J, Alzate-Granados JP, Rojas-Villarraga A
Adv Rheumatol 2021 Feb 3;61(1):8. doi: 10.1186/s42358-020-00158-0. PMID: 33536075
Diagnostic value of labial minor salivary gland biopsy for Sjögren's syndrome: a systematic review.
Guellec D, Cornec D, Jousse-Joulin S, Marhadour T, Marcorelles P, Pers JO, Saraux A, Devauchelle-Pensec V
Autoimmun Rev 2013 Jan;12(3):416-20. Epub 2012 Aug 7 doi: 10.1016/j.autrev.2012.08.001. PMID: 22889617

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