ERN ReCONNET

Disease Info

Disease: Sjögren’s Disease

Sjögren’s Disease (SjD)

Sjögren’s Disease (SjD) 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 disease 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 disease mostly occurs in middle-aged women.

The diagnosis of Sjögren’s disease 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.

 

Gaetane Nocturne

Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre

Gaetane Nocturne
Disease Coordinator

Ana Vieira

Liga Portuguesa Contra as Doenças Reumáticas

Ana Vieira
ePAG representative

Patients' Organisations

Patients’ Organisations

Chiara Baldini

AOUP Pisa, Italy

Chiara Baldini
Disease Coordinator

Coralie Bouillot

Sjögren Europe

Coralie Bouillot
ePAG representative

 

 

 

ERN ReCONNET Infographic on Videocapillaroscopy

The ERN ReCONNET Education and Training Working Group (WG) is glad to release its new flyer on nailfold videocapillaroscopy (NVC), a new and informative document targeting patients and lay audience aimed at explaining such an important diagnostic approach.
Regarding the NVC approach, the test is mainly useful in rheumatology and in internal medicine, for an early or to-be-confirmed diagnosis of rare and complex connective tissue diseases such as System Sclerosis (SSc),  Polydermatomyositis, Mixed Connective Tissue Disease (MCTD), Undifferentiated Connective Tissue Disease (UCTD), AntiPhospholipid Syndrome (APS), and offers important information on microcirculation in Systemic Lupus Erythematosus (SLE) as well as in Sjögren’s Disease (SjD) and overlaps.
The flyer can be downloaded for free (pdf), and it will soon available in several languages:
ERN ReCONNET Capillaroscopy Flyer (pdf)

More details and info about NVC can be found here.

WEBINARS ON SS ARE FREELY AVAILABLE

Sjögren’s essentials. Dr. Vasco Romao.

The oral component of Sjögren’s disease. Prof. Arjan Vissink.

How to explain Sjögren's Disease to your family and friends. Prof. Eric Hachulla - ePAGs Ana Vieira and Coralie Bouillot.

ePAG Coralie Bouillot video messages

ERN ReCONNET @ EULAR2023: ePAG's Coraline Bouillot opinion (FRENCH)

ERN ReCONNET @ EULAR2023: ePAG's Coraline Bouillot opinion (ENGLISH)

ERN ReCONNET publications are available for consultation.

LIST OF USEFUL REFERENCES

Currently existing evidence-based guidelines

  1. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).
    Antoniou KM, Distler O, Gheorghiu AM, Moor CC, Vikse J, Bizymi N, Galetti I, Brown G, Bargagli E, Allanore Y, Corte TJ, Dieudé P, Cottin V, Fisher BA, Fabre A, Giles JT, Kreuter M, Lundberg IE, Poletti V, Maurer B, Renzoni EA, Müller-Ladner U, Strek ME, Sverzellati N, Studenic P, Mohammed J, Nagavci B, Stamm T, Tonia T, Crestani B, Hoffmann-Vold AM.
    Ann Rheum Dis. 2026 Jan;85(1):22-60. doi: 10.1016/j.ard.2025.08.021. Epub 2025 Sep 4. PMID: 40912974.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.
  12. 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.
  13. 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.
  14. 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.
  15. 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.

Classification criteria and disease-specific tools

  1. Recommendations for Aligned Nomenclature of Peripheral Nervous System Disorders Across Rheumatology and Neurology
    Noaiseh G, Deboo A, King JK, Varadhachary A, Sarka G, Goodman BP, Hammitt KM, Frantsve-Hawley J, Fox R, Baker MC, Danielides S, Mandel S, Pavlakis PP, Scofield RH, Wallace DJ, Carteron N, Carsons S.
    Arthritis Rheumatol. 2025 Apr;77(4):383-389. doi: 10.1002/art.43050. Epub 2025 Jan 6. PMID: 39489692; PMCID: PMC11936496
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.

Other important reviews and recommendations

  1. International Task Force on Nomenclature of Sjögren Disease. 2023 International Rome consensus for the nomenclature of Sjögren disease
    Ramos-Casals M, Baer AN, Brito-Zerón MDP, Hammitt KM, Bouillot C, Retamozo S, Mackey A, Yarowsky D, Turner B, Blanck J, Fisher BA, Akpek EK, Baldini C, Bootsma H, Bowman SJ, Dörner T, Laing L, Lieberman SM, Mariette X, Pflugfelder SC, Sankar V, Sisó-Almirall A, Tzioufas AG, Anaya JM, Armağan B, Bombardieri M, Carsons S, de Vita S, Fox RI, Gerli R, Giacomelli R, Gottenberg JE, Hernández-Molina G, Jonsson R, Kruize A, Kwok SK, Li X, McCoy SS, Ng WF, Olsson P, Rischmueller M, Saraux A, Scofield RH, Valim V, Vitali C, Vivino F, Wahren-Herlenius M, Moutsopoulos HM
    Nat Rev Rheumatol. 2025 Jul;21(7):426-437. doi: 10.1038/s41584-025-01268-z. Epub 2025 Jun 10. PMID: 40494962
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. Treatment of primary Sjögren syndrome
    Saraux A, Pers JO, Devauchelle-Pensec V.
    Nat Rev Rheumatol. 2016 Aug;12(8):456-71.
  7. 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.
  8. 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.
  9. 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.
  10. 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.

SjD centres in ERN ReCONNET

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