Download PDF
Aktuelle Rheumatologie 2019; 44(02): 102-108
DOI: 10.1055/s-0043-119413
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Sjögren-Syndrom bei Kindern und Jugendlichen

Juvenile Sjögren’s Syndrome
Johannes-Peter Haas
1   Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen
› Author Affiliations
Further Information

Publication History

Publication Date:
13 February 2018 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Das Sjögren Syndrom ist bei jungen Erwachsenen eine seltene, jedoch oft zu spät erkannte Autoimmunopathie. Es manifestiert sich als primäres juveniles Sjögren Syndrom (jSS) oder im Rahmen anderer Autoimmunerkrankungen als sekundäres jSS. Die Klinik weicht in einigen Aspekten von der des SS beim Adulten ab. Differentialdiagnosen sind andere Formen der Parotitis, die bei Kindern und Jugendlichen häufiger vorkommen. Schwere systemische Verläufe eines jSS und selbst die Entwicklung von B-Zell-Lymphomen werden jedoch auch bei jungen Patienten beobachtet. Daher sollte auch bei Jugendlichen mit entsprechenden Erstsymptomen an ein jSS gedacht und eine entsprechende Diagnostik veranlasst werden. Patienten mit einem jSS bedürfen einer regelmäßigen kinderrheumatologischen Betreuung.

Abstract

Sjögren’s syndrome in adolescents is a rare autoimmune disease, which is often diagnosed with delay. It may present as a primary juvenile Sjögren’s syndrome (jSS) or in association with other autoimmune diseases as a secondary form of jSS. Differential diagnoses include other forms of parotitis, which are more frequent in that age group. The clinical presentation of jSS differs from SS in adults in several aspects. However, severe systemic illness and even the development of B-cell lymphoma have also been observed in young patients suffering from jSS. Therefore, jSS should be considered as a diagnosis in cases of recurrent sialadenitis in adolescents if there are other suspicious findings. Early diagnosis as well as tight monitoring and treatment by paediatric rheumatologists are mandatory in patients with jSS in order to control disease activity and to minimise long-term damage.

Schlüsselwörter

Juvenile - Sjögren Syndrom - Adoleszenz - Kollagenose - Kinderrheumatologie

Key words

Juvenile - mixed connective tissue disease - children - Sjögren's syndrome - paediatric rheumatology
 

  • Literatur

  • 1 Vitali C, Bombardieri S, Jonsson R. et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Annals of the rheumatic diseases 2002; 61: 554-5558
    CrossrefPubMedGoogle Scholar
  • 2 Shiboski CH, Shiboski SC, Seror R. et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren's syndrome: A consensus and data-driven methodology involving three international patient cohorts. Annals of the rheumatic diseases 2017; 76: 9-16
    CrossrefPubMedGoogle Scholar
  • 3 Bartunkova J, Sediva A, Vencovsky J. et al. Primary Sjogren's syndrome in children and adolescents: proposal for diagnostic criteria. Clinical and experimental rheumatology 1999; 17: 381-386
    PubMedGoogle Scholar
  • 4 Yokogawa N, Lieberman SM, Sherry DD. et al. Features of childhood Sjogren's syndrome in comparison to adult Sjogren's syndrome: considerations in establishing child-specific diagnostic criteria. Clinical and experimental rheumatology 2016; 34: 343-351
    PubMedGoogle Scholar
  • 5 Lieberman SM. Childhood Sjogren syndrome: insights from adults and animal models. Current opinion in rheumatology 2013; 25: 651-657
    CrossrefPubMedGoogle Scholar
  • 6 Ambrus JL, Suresh L, Peck A. Multiple Roles for B-Lymphocytes in Sjogren's Syndrome. Journal of clinical medicine 2016; 5:
    PubMed
  • 7 Maslinska M, Przygodzka M, Kwiatkowska B. et al. Sjogren's syndrome: still not fully understood disease. Rheumatology international 2015; 35: 233-241
    CrossrefPubMedGoogle Scholar
  • 8 Varoczy L, Gergely L, Zeher M. et al. Malignant lymphoma-associated autoimmune diseases – a descriptive epidemiological study. Rheumatology international 2002; 22: 233-237
    CrossrefPubMedGoogle Scholar
  • 9 Hofauer B, Mansour N, Heiser C. et al. Sonoelastographic Modalities in the Evaluation of Salivary Gland Characteristics in Sjogren's Syndrome. Ultrasound in medicine & biology 2016; 42: 2130-2139
    CrossrefPubMedGoogle Scholar
  • 10 Krumrey-Langkammerer M, Haas JP. Wertigkeit der Speicheldrüsen-Sonografie bei Sjögrensyndrom im Kindes-, und Jugendalter. Ultraschall in Med 2015; 36: A349
    Article in Thieme ConnectPubMedGoogle Scholar
  • 11 Fisher BA, Jonsson R, Daniels T. et al. Standardisation of labial salivary gland histopathology in clinical trials in primary Sjogren's syndrome. Annals of the rheumatic diseases 2016; DOI: 10.1136/annrheumdis-2016-210448.
    CrossrefPubMedGoogle Scholar
  • 12 Bouayed K, Alyanakian MA, Cordonnier C. et al. Sjogren syndrome: an unexpected disease occurring fourteen years after oligoarticular onset juvenile idiopathic arthritis. Rheumatology (Oxford, England) 2002; 41: 1190-1191
    CrossrefPubMedGoogle Scholar
  • 13 Kuo CF, Grainge MJ, Valdes AM. et al. Familial Risk of Sjogren's Syndrome and Co-aggregation of Autoimmune Diseases in Affected Families: A Nationwide Population Study. Arthritis & rheumatology (Hoboken, NJ) 2015; 67: 1904-1912
    CrossrefPubMedGoogle Scholar
  • 14 Neiman AR, Lee LA, Weston WL. et al. Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry. The Journal of pediatrics 2000; 137: 674-680
    CrossrefPubMedGoogle Scholar
  • 15 Yadlapati S, Efthimiou P. Autoimmune/Inflammatory Arthritis Associated Lymphomas: Who Is at Risk?. BioMed research international 2016; 2016: 8631061
    PubMedGoogle Scholar
  • 16 Aoun G, Nasseh I, Berberi A. Evaluation of the oral component of Sjogren's syndrome: An overview. Journal of International Society of Preventive & Community Dentistry 2016; 6: 278-284
    PubMedGoogle Scholar
  • 17 Kleger A, Seufferlein T, Wagner M. et al. IgG4-associated autoimmune diseases—polymorphous presentation complicates diagnosis and treatment. Dtsch Arztebl Int 2015; 112: 128-135
    PubMedGoogle Scholar
  • 18 Whitcher JP, Shiboski CH, Shiboski SC. et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjogren's Syndrome International Registry. American journal of ophthalmology 2010; 149: 405-415
    CrossrefPubMedGoogle Scholar
  • 19 van Bijsterveld OP, Mackor AJ. Sjogren's syndrome and tear function parameters. Clinical and experimental rheumatology 1989; 7: 151-154
    PubMedGoogle Scholar
  • 20 Lin D, Yang W, Guo X. et al. Cross-sectional comparison of ultrasonography scoring systems for primary Sjogren's syndrome. International journal of clinical and experimental medicine 2015; 8: 19065-19071
    PubMedGoogle Scholar
  • 21 Jousse-Joulin S, Milic V, Jonsson MV. et al. Is salivary gland ultrasonography a useful tool in Sjogren's syndrome? A systematic review. Rheumatology (Oxford, England) 2016; 55: 789-800
    CrossrefPubMedGoogle Scholar
  • 22 Hocevar A, Ambrozic A, Rozman B. et al. Ultrasonographic changes of major salivary glands in primary Sjogren's syndrome. Diagnostic value of a novel scoring system. Rheumatology (Oxford, England) 2005; 44: 768-772
    PubMedGoogle Scholar
  • 23 Hocevar A, Rainer S, Rozman B. et al. Ultrasonographic changes of major salivary glands in primary Sjogren's syndrome. Evaluation of a novel scoring system. European journal of radiology 2007; 63: 379-383
    CrossrefPubMedGoogle Scholar
  • 24 Akasbi M, Berenguer J, Saiz A. et al. White matter abnormalities in primary Sjogren syndrome. QJM: monthly journal of the Association of Physicians 2012; 105: 433-443
    CrossrefPubMedGoogle Scholar
  • 25 Zhu L, Zhang C, Hua Y. et al. Dynamic contrast-enhanced MR in the diagnosis of lympho-associated benign and malignant lesions in the parotid gland. Dento maxillo facial radiology 2016; 45: 20150343
    CrossrefPubMedGoogle Scholar
  • 26 Ramos-Casals M, Tzioufas AG, Stone JH. et al. Treatment of primary Sjogren syndrome: a systematic review. Jama 2010; 304: 452-460
    CrossrefPubMedGoogle Scholar
  • 27 Moscovici BK, Holzchuh R, Sakassegawa-Naves FE. et al. Treatment of Sjogren's syndrome dry eye using 0.03% tacrolimus eye drop: Prospective double-blind randomized study. Contact lens & anterior eye: the journal of the British Contact Lens Association 2015; 38: 373-378
    CrossrefPubMedGoogle Scholar
  • 28 Tomiita M, Takei S, Kuwada N. et al. Efficacy and safety of orally administered pilocarpine hydrochloride for patients with juvenile-onset Sjogren's syndrome. Modern rheumatology 2010; 20: 486-490
    CrossrefPubMedGoogle Scholar
  • 29 Pereira PR, Viala K, Maisonobe T. et al. Sjogren Sensory Neuronopathy (Sjogren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases. Medicine 2016; 95: e3632
    PubMedGoogle Scholar
  • 30 Skopouli FN, Jagiello P, Tsifetaki N. et al. Methotrexate in primary Sjogren's syndrome. Clinical and experimental rheumatology 1996; 14: 555-558
    PubMedGoogle Scholar
  • 31 Ozgocmen S, Gur A. Treatment of central nervous system involvement associated with primary Sjogren's syndrome. Current pharmaceutical design 2008; 14: 1270-1273
    CrossrefPubMedGoogle Scholar
  • 32 Ueda Y, Tomoe H, Takahashi H. et al. Interstitial cystitis associated with primary Sjogren's syndrome successfully treated with a combination of tacrolimus and corticosteroid: A case report and literature review. Modern rheumatology 2016; 26: 445-449
    CrossrefPubMedGoogle Scholar
  • 33 Fox RI, Dixon R, Guarrasi V. et al. Treatment of primary Sjogren's syndrome with hydroxychloroquine: a retrospective, open-label study. Lupus 1996; 5 (Suppl. 01) S31-S36
    CrossrefPubMedGoogle Scholar
  • 34 Gottenberg JE, Ravaud P, Puechal X. et al. Effects of hydroxychloroquine on symptomatic improvement in primary Sjogren syndrome: the JOQUER randomized clinical trial. Jama 2014; 312: 249-258
    CrossrefPubMedGoogle Scholar
  • 35 Evans RD, Laing CM, Ciurtin C. et al. Tubulointerstitial nephritis in primary Sjogren syndrome: clinical manifestations and response to treatment. BMC musculoskeletal disorders 2016; 17: 2
    CrossrefPubMedGoogle Scholar
  • 36 Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest 2013; 143: 814-824
    CrossrefPubMedGoogle Scholar
  • 37 Dass S, Bowman SJ, Vital EM. et al. Reduction of fatigue in Sjogren syndrome with rituximab: results of a randomised, double-blind, placebo-controlled pilot study. Annals of the rheumatic diseases 2008; 67: 1541-1544
    CrossrefPubMedGoogle Scholar
  • 38 Voulgarelis M, Ziakas PD, Papageorgiou A. et al. Prognosis and outcome of non-Hodgkin lymphoma in primary Sjogren syndrome. Medicine 2012; 91: 1-9
    PubMedGoogle Scholar
  • 39 Tsuboi H, Matsumoto I, Hagiwara S. et al. Efficacy and safety of abatacept for patients with Sjogren's syndrome associated with rheumatoid arthritis: rheumatoid arthritis with orencia trial toward Sjogren's syndrome Endocrinopathy (ROSE) trial-an open-label, one-year, prospective study-Interim analysis of 32 patients for 24 weeks. Modern rheumatology 2015; 25: 187-193
    CrossrefPubMedGoogle Scholar
  • 40 Haacke EA, van der Vegt B, Meiners PM et al. Abatacept treatment of patients with primary Sjogren's syndrome results in a decrease of germinal centres in salivary gland tissue. Clinical and experimental rheumatology 2016 DOI
    PubMed
  • 41 Quartuccio L, Salvin S, Corazza L. et al. Efficacy of belimumab and targeting of rheumatoid factor-positive B-cell expansion in Sjogren's syndrome: follow-up after the end of the phase II open-label BELISS study. Clinical and experimental rheumatology 2016; 34: 311-314
    PubMedGoogle Scholar
  • 42 Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjogren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford, England) 2016; 55: 450-460
    PubMedGoogle Scholar
  • 43 Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use. Medicine 2016; 95: e3766
    PubMedGoogle Scholar
  • 44 Gritzmann N. Ultrasound of the salivary glands. Laryngo- rhino- otologie 2009; 88: 48-56 quiz 57-49
    Article in Thieme ConnectPubMedGoogle Scholar