ERN ReCONNET

Disease Info

Disease: Sjögren’s syndrome

Sjögren’s syndrome

Sjögren’s syndrome 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.

Disease_SS
Disease_SS

LIST OF USEFUL REFERENCES

Currently existing evidence-based guidelines

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  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.

Classification criteria and disease-specific tools

  • 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.

    • Other important reviews and recommendations

  • 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.
  • 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.
  • 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.
  • 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.
  • 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.
  • Treatment of primary Sjögren syndrome
    Saraux A, Pers JO, Devauchelle-Pensec V.
    Nat Rev Rheumatol. 2016 Aug;12(8):456-71.
  • 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.

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