ERN ReCONNET
Disease Info
Disease: Sjögren’s syndrome
Sjögren’s syndrome (SS)
Sjögren’s syndrome (SS) is a systemic autoimmune disease that involves particularly salivary and lachrymal glands. As a consequence, typically, patients complain of ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are quite common as well. In addition to dryness, other frequently reported symptoms are also widespread pain and fatigue. In one third of the patients, Sjögren’s syndrome may also affect other parts of the body, including your joints, lungs, kidneys, blood vessels, digestive organs, and nerves. A minority of patients may develop a non Hodgkin’s lymphoma as a complication of the disease. Sjögren’s syndrome mostly occurs in middle-aged women.
The diagnosis of Sjögren’s syndrome is made on the basis of medical history, physical exam, ocular and oral tests, blood tests, and salivary gland biopsies. Treatment focuses on relieving symptoms by using artificial tears, sugar-free candy, drinking water often for a dry mouth or an oral drug called pilocarpine that may improve dryness. Extra glandular symptoms may require immunosuppressive drugs. Novel biological drugs are in the pipeline for the treatment of the disease.
ERN ReCONNET publications are available for consultation.
As ERN ReCONNET, we are happy to support our Sjögren’s syndrome patient community by joining the forces to raise
awareness together on its awareness day.
WEBINARS
awareness together on its awareness day.
Xavier Mariette
Xavier Mariette
Disease Coordinator
Disease Coordinator
Ana Vieira
ePAG representative
ePAG representative
Vasco Romão
Vasco Romão
Disease Coordinator
Disease Coordinator
Coralie Bouillot
ePAG representative
ePAG representative
LIST OF USEFUL REFERENCES
- Practical guidelines for the early diagnosis of Sjögren’s syndrome in primary healthcare
Sisó-Almirall A, Meijer JM, Brito-Zerón P, Conangla L, Flores-Chavez A, González de Paz L, Bootsma H, Ramos-Casals M.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):197-205.
- The management of Sjögren’s syndrome: British Society for Rheumatology guideline scope
Price E, Allen A, Rauz S, Tappuni A, Sutcliffe N, Bombardieri M, Carty S, Ciurtin C, Crampton B, Duncalfe L, Fisher B, Glennon P, Hackett KL, Larkin G, Ng WF, Ramanan AV, Rassam S, Walsh SB, Bowman S.
Rheumatology (Oxford). 2021 May 14;60(5):2122-2127.
- Therapeutic Recommendations for the Management of Older Adult Patients with Sjögren’s Syndrome
Retamozo S, Baldini C, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernández-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vissink A, Vitali C, Bowman SJ, Mariette X, Ramos-Casals M, Brito-Zerón P.
Drugs Aging. 2021 Apr;38(4):265-284.
- Clinical practice guidance for Sjögren’s syndrome in pediatric patients (2018) – summarized and updated
Tomiita M, Kobayashi I, Itoh Y, Inoue Y, Iwata N, Umebayashi H, Okamoto N, Nonaka Y, Hara R, Mori M.
Mod Rheumatol. 2021 Mar;31(2):283-293.
- Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren’s
Lee AS, Scofield RH, Hammitt KM, Gupta N, Thomas DE, Moua T, Ussavarungsi K, St Clair EW, Meehan R, Dunleavy K, Makara M, Carsons SE, Carteron NL; Consensus Expert Panel (CEP) Members
Chest. 2021 Feb;159(2):683-698.
- 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, et al. On behalf of the EULAR-Sjögren Syndrome Task Force Group
Ann Rheum Dis. 2020;79:3-18.
- The British Society for Rheumatology guideline for the management of adults with primary Sjögren’s Syndrome
Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, et al.
Rheumatology (Oxford). 2017 Oct 1; 56(10):1828.
- Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain
Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.
Arthritis Care Res. 2017 Apr;69(4):517-27.
- New Treatment Guidelines for Sjögren’s Disease
Vivino FB, Carsons SE, Foulks G, Daniels TE, Parke A, Brennan MT, et al.
Rheum Dis Clin North Am. 2016 Aug;42(3):531-51.
- Clinical practice guidelines for oral management of Sjögren disease: Dental caries prevention
Zero DT, Brennan MT, Daniels TE, Papas A, Stewart C, Pinto A, et al.
J Am Dent Assoc. 2016 Apr;147(4):295-305.
- Early diagnosis of primary Sjögren’s syndrome: EULAR-SS task force clinical recommendations
Brito-Zerón P, Theander E, Baldini C, Seror R, Retamozo S, Quartuccio L, et al.
Expert Rev Clin Immunol. 2016;12(2):137-56.
- Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements
Ramos-Casals M, Brito-Zerón P, Seror R, Bootsma H, Bowman SJ, Dörner T, et al.
Rheumatology (Oxford). 2015 Dec;54(12):2230-8.
- Recommendations for the treatment of Sjögren’s syndrome
Valim V, Trevisani VFM, Pasoto SG, Serrano EV, Ribeiro SLE, de Alencar Fidelix TS, et al.
Rev Bras Reumatol (English Ed). 2015 Sep-Oct;55(5):446-57.
- Clinical guidelines for management of dry eye associated with Sjögren disease
Foulks GN, Forstot SL, Donshik PC, Forstot JZ, Goldstein MH, Lemp MA, et al.
Ocul Surf. 2015 Apr;13(2):118-32.
Sisó-Almirall A, Meijer JM, Brito-Zerón P, Conangla L, Flores-Chavez A, González de Paz L, Bootsma H, Ramos-Casals M.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):197-205.
Price E, Allen A, Rauz S, Tappuni A, Sutcliffe N, Bombardieri M, Carty S, Ciurtin C, Crampton B, Duncalfe L, Fisher B, Glennon P, Hackett KL, Larkin G, Ng WF, Ramanan AV, Rassam S, Walsh SB, Bowman S.
Rheumatology (Oxford). 2021 May 14;60(5):2122-2127.
Retamozo S, Baldini C, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernández-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vissink A, Vitali C, Bowman SJ, Mariette X, Ramos-Casals M, Brito-Zerón P.
Drugs Aging. 2021 Apr;38(4):265-284.
Tomiita M, Kobayashi I, Itoh Y, Inoue Y, Iwata N, Umebayashi H, Okamoto N, Nonaka Y, Hara R, Mori M.
Mod Rheumatol. 2021 Mar;31(2):283-293.
Lee AS, Scofield RH, Hammitt KM, Gupta N, Thomas DE, Moua T, Ussavarungsi K, St Clair EW, Meehan R, Dunleavy K, Makara M, Carsons SE, Carteron NL; Consensus Expert Panel (CEP) Members
Chest. 2021 Feb;159(2):683-698.
Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, et al. On behalf of the EULAR-Sjögren Syndrome Task Force Group
Ann Rheum Dis. 2020;79:3-18.
Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, et al.
Rheumatology (Oxford). 2017 Oct 1; 56(10):1828.
Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.
Arthritis Care Res. 2017 Apr;69(4):517-27.
Vivino FB, Carsons SE, Foulks G, Daniels TE, Parke A, Brennan MT, et al.
Rheum Dis Clin North Am. 2016 Aug;42(3):531-51.
Zero DT, Brennan MT, Daniels TE, Papas A, Stewart C, Pinto A, et al.
J Am Dent Assoc. 2016 Apr;147(4):295-305.
Brito-Zerón P, Theander E, Baldini C, Seror R, Retamozo S, Quartuccio L, et al.
Expert Rev Clin Immunol. 2016;12(2):137-56.
Ramos-Casals M, Brito-Zerón P, Seror R, Bootsma H, Bowman SJ, Dörner T, et al.
Rheumatology (Oxford). 2015 Dec;54(12):2230-8.
Valim V, Trevisani VFM, Pasoto SG, Serrano EV, Ribeiro SLE, de Alencar Fidelix TS, et al.
Rev Bras Reumatol (English Ed). 2015 Sep-Oct;55(5):446-57.
Foulks GN, Forstot SL, Donshik PC, Forstot JZ, Goldstein MH, Lemp MA, et al.
Ocul Surf. 2015 Apr;13(2):118-32.
- 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al.
Ann Rheum Dis. 2017 Jan;76(1):9-16.
- American College of Rheumatology Classification Criteria for Sjögren’s Syndrome: A Data-Driven, Expert Consensus Approach in the Sjögren’s International Collaborative Clinical Alliance cohort
Shiboski SC, Shiboski CH, Criswell LA, Baer AN, Challacombe S, Lanfranchi H, et al.
Arthritis Care Res (Hoboken). 2012 Apr;64(4):475-87.
- Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al.
Ann Rheum Dis. 2002 Jun;61(6):554-8.
- EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjögren’s syndrome
Seror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A, et al.
Ann Rheum Dis. 2011 Jun;70(6):968-72.
- EULAR Sjögren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome
Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, et al.
Ann Rheum Dis. 2010 Jun;69(6):1103-9.
- Development of the ClinESSDAI: a clinical score without biological domain. A tool for biological studies
Seror R, Meiners P, Baron G, Bootsma H, Bowman SJ, Vitali C, et al, on behalf of the EULAR Sjögren’s Task Force.
Ann Rheum Dis. 2016 Nov;75(11):1945-1950.
- EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide
Seror R, Bowman SJ, Brito-Zerón P, Theander E, Bootsma H, Tzioufas A, et al, on behalf of the EULAR Sjögren’s Task Force
RMD Open. 2015;1:e000022.
- Defining disease activity states and clinically meaningful improvement in primary Sjögren’s syndrome with EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)
Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG, et al, on behalf of the EULAR Sjögren’s Task Force
Ann Rheum Dis. 2016 Feb;75(2):382-9.
- Sjögren’s Syndrome Disease Damage Index and Disease Activity Index. Scoring Systems for the Assessment of Disease Damage and Disease Activity in Sjögren’s Syndrome, Derived From an Analysis of a Cohort of Italian Patients
Vitali C, Palombi G, Baldini C, Benucci M, Bombardieri S, Covelli M, et al.
Arthritis Rheum. 2007 Jul;56(7):2223-31.
- The Sjögren’s Syndrome Damage Index—a damage index for use in clinical trials and observational studies in primary Sjögren’s syndrome
Barry RJ, Sutcliffe N, Isenberg DA, Price E, Goldblatt F, Adler M, et al.
Rheumatology (Oxford). 2008 Aug;47(8):1193-8.
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al.
Ann Rheum Dis. 2017 Jan;76(1):9-16.
Shiboski SC, Shiboski CH, Criswell LA, Baer AN, Challacombe S, Lanfranchi H, et al.
Arthritis Care Res (Hoboken). 2012 Apr;64(4):475-87.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al.
Ann Rheum Dis. 2002 Jun;61(6):554-8.
Seror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A, et al.
Ann Rheum Dis. 2011 Jun;70(6):968-72.
Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, et al.
Ann Rheum Dis. 2010 Jun;69(6):1103-9.
Seror R, Meiners P, Baron G, Bootsma H, Bowman SJ, Vitali C, et al, on behalf of the EULAR Sjögren’s Task Force.
Ann Rheum Dis. 2016 Nov;75(11):1945-1950.
Seror R, Bowman SJ, Brito-Zerón P, Theander E, Bootsma H, Tzioufas A, et al, on behalf of the EULAR Sjögren’s Task Force
RMD Open. 2015;1:e000022.
Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG, et al, on behalf of the EULAR Sjögren’s Task Force
Ann Rheum Dis. 2016 Feb;75(2):382-9.
Vitali C, Palombi G, Baldini C, Benucci M, Bombardieri S, Covelli M, et al.
Arthritis Rheum. 2007 Jul;56(7):2223-31.
Barry RJ, Sutcliffe N, Isenberg DA, Price E, Goldblatt F, Adler M, et al.
Rheumatology (Oxford). 2008 Aug;47(8):1193-8.
- Differences in presentation between paediatric- and adult-onset primary Sjögren’s syndrome patients
Legger GE, Erdtsieck MB, de Wolff L, Stel AJ, Los LI, Verstappen GM, Spijkervet FKL, Vissink A, van der Vegt B, Kroese FGM, Armbrust W, Arends S, Bootsma H.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):85-92.
- Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium
Retamozo S, Acar-Denizli N, Horváth IF, Ng WF, Rasmussen A, Dong X, Li X, Baldini C, Olsson P, Priori R, Seror R, Gottenberg JE, Kruize AA, Hernandez-Molina G, Vissink A, Sandhya P, Armagan B, Quartuccio L, Sebastian A, Praprotnik S, Bartoloni E, Kwok SK, Kvarnstrom M, Rischmueller M, Soláns-Laqué R, Sene D, Pasoto SG, Suzuki Y, Isenberg DA, Valim V, Nordmark G, Nakamura H, Fernandes Moça Trevisani V, Hofauer B, Sisó-Almirall A, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Atzeni F, Hammenfors D, Maure B, Carsons SE, Gheita T, Sánchez-Berná I, López-Dupla M, Morel J, Inanç N, Fonseca-Aizpuru E, Morcillo C, Vollenweider C, Melchor S, Vázquez M, Díaz-Cuiza E, Consani-Fernández S, de-Miguel-Campo B, Szántó A, Bombardieri S, Gattamelata A, Hinrichs A, Sánchez-Guerrero J, Danda D, Kilic L, De Vita S, Wiland P, Gerli R, Park SH, Wahren-Herlenius M, Bootsma H, Mariette X, Ramos-Casals M, Brito-Zerón P; Sjögren Big Data Consortium.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):166-174.
- Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren’s syndrome
Fisher BA, Jonsson R, Daniels T, Bombardieri M, Brown RM, Morgan P, et al.
Ann Rheum Dis. 2017 Jul;76(7):1161-8.
- Salivary gland ultrasound abnormalities in primary Sjögren’s syndrome: consensual US-SG core items definition and reliability
Jousse-Joulin S, Nowak E, Cornec D, Brown J, Carr A, Carotti M, et al.
RMD Open 2017 Jun 9;3(1):e000364.
- Treatment of primary Sjögren syndrome
Saraux A, Pers JO, Devauchelle-Pensec V.
Nat Rev Rheumatol. 2016 Aug;12(8):456-71.
- Use of Biologics in Sjögren’s Syndrome
Nocturne G, Cornec D, Seror R, Mariette X.
Rheum Dis Clin North Am. 2016 Aug;42(3):407-17.
- Sjögren syndrome
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al.
Nat Rev Dis Primers. 2016 Jul 7;2:16047.
- Treatment of Sjögren’s Syndrome-associated dry eye: An evidence-based review
Akpek EK, Lindsley KB, Adyanthaya RS, Swamy R, Baer AN, McDonnell PJ.
Ophthalmology. 2011 Jul;118(7):1242-52.
- Salivary dysfunction associated with systemic diseases: systematic review and clinical management recommendations
von Bültzingslöwen I, Sollecito TP, Fox PC, Daniels T, Jonsson R, Lockhart PB, et al.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Mar;103 Suppl:S57.e1-15.
Legger GE, Erdtsieck MB, de Wolff L, Stel AJ, Los LI, Verstappen GM, Spijkervet FKL, Vissink A, van der Vegt B, Kroese FGM, Armbrust W, Arends S, Bootsma H.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):85-92.
Retamozo S, Acar-Denizli N, Horváth IF, Ng WF, Rasmussen A, Dong X, Li X, Baldini C, Olsson P, Priori R, Seror R, Gottenberg JE, Kruize AA, Hernandez-Molina G, Vissink A, Sandhya P, Armagan B, Quartuccio L, Sebastian A, Praprotnik S, Bartoloni E, Kwok SK, Kvarnstrom M, Rischmueller M, Soláns-Laqué R, Sene D, Pasoto SG, Suzuki Y, Isenberg DA, Valim V, Nordmark G, Nakamura H, Fernandes Moça Trevisani V, Hofauer B, Sisó-Almirall A, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Atzeni F, Hammenfors D, Maure B, Carsons SE, Gheita T, Sánchez-Berná I, López-Dupla M, Morel J, Inanç N, Fonseca-Aizpuru E, Morcillo C, Vollenweider C, Melchor S, Vázquez M, Díaz-Cuiza E, Consani-Fernández S, de-Miguel-Campo B, Szántó A, Bombardieri S, Gattamelata A, Hinrichs A, Sánchez-Guerrero J, Danda D, Kilic L, De Vita S, Wiland P, Gerli R, Park SH, Wahren-Herlenius M, Bootsma H, Mariette X, Ramos-Casals M, Brito-Zerón P; Sjögren Big Data Consortium.
Clin Exp Rheumatol. 2021 Nov-Dec;39 Suppl 133(6):166-174.
Fisher BA, Jonsson R, Daniels T, Bombardieri M, Brown RM, Morgan P, et al.
Ann Rheum Dis. 2017 Jul;76(7):1161-8.
Jousse-Joulin S, Nowak E, Cornec D, Brown J, Carr A, Carotti M, et al.
RMD Open 2017 Jun 9;3(1):e000364.
Saraux A, Pers JO, Devauchelle-Pensec V.
Nat Rev Rheumatol. 2016 Aug;12(8):456-71.
Nocturne G, Cornec D, Seror R, Mariette X.
Rheum Dis Clin North Am. 2016 Aug;42(3):407-17.
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al.
Nat Rev Dis Primers. 2016 Jul 7;2:16047.
Akpek EK, Lindsley KB, Adyanthaya RS, Swamy R, Baer AN, McDonnell PJ.
Ophthalmology. 2011 Jul;118(7):1242-52.
von Bültzingslöwen I, Sollecito TP, Fox PC, Daniels T, Jonsson R, Lockhart PB, et al.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Mar;103 Suppl:S57.e1-15.
SS centres in ERN ReCONNET
Helsinki University Hospital, Hospital District of Helsinki and Uusimaa, Finland (ReCONNETFIN) (adult and paedriatric)
- Civil Hospital – Brescia
- AOU Careggi, Florence
- AOU Meyer di Firenze (paediatric)
- Foundation IRCCS CA’Granda Ospedale Maggiore polyclinic – Milan
- ASST Centro Specialistico Ortopedico Traumatologico Gaetano Pini (Presidio Pini)
- AOU Pisan
- AOU Policlinico Umberto I di Roma
- Fondazione Policlinico Tor Vergata Roma
- AOU S. Giovanni di Dio e Ruggi D’Aragona
- A.S.L. Torino – Hub S. Giovanni Bosco (paediatric)
- Azienda Sanitaria Universitaria Friuli Centrale (ex ASUIUD ora ASUFC) (adult and paediatric)
- Azienda Ospedaliera Universitaria Integrata di Verona
- Hospital Clínic de Barcelona y Hospital de Sant Joan de Déu (adult and paediatric)
- Hospital Universitari Vall d’Hebron (adult and paediatric)
- Hospital Universitario 12 de Octubre
