Komplikationen beim Sjögren Syndrom

Zusammenfassung

Das Sjögren-Syndrom ist eine entzündlichrheumatische Erkrankung, die zu einer Reihe von Komplikationen im internistischen Bereich führen kann, insbesondere zu erhöhten Risiken für Non-Hodgkin-Lymphome, Vaskulitiden, zu erhöhter Infektionsneigung, Immunzytopenien und Thromboseneigung. In der Geburtshilfe zeigen sich vermehrt fetomaternale Syndrome wie angeborener Herzblock oder neonataler Lupus erythematodes, während im Bereich der Neurologie u. a. sensible Neuronopathie und Demyelinisierungen vorkommen können. Aktuelle Aspekte in der Patientenführung durch den Rheumatologen mit Bezug auf diagnostische, therapeutische als auch prognostische Aspekte werden in dieser Übersicht dargestellt. Obgleich es in der Behandlung keine spezifischen Empfehlungen für Sjögren-Patienten gibt, stellt das Management der Komplikationen bei dieser Erkrankung zweifelsohne erneut heraus, dass hier ein interdisziplinärer Zugang von herausragender Stellung vonnöten ist.

Summary

Sjögren’s syndrome is considered an inflammatory rheumatic disease and part of connective tissue diseases with a number of clinical complications. In particular, Sjögren’s has a substantially increased risk of developing Non-Hodgkin’s lymphoma, while a substantial number of patients develop vasculitides, infections, immune cytopenia, venous thrombembolic and obstetric complications (i. e. fetomaternale autoimmune syndromes, such as congenital heart block, neonatal lupus), peridontal as well as neurologic manifestations (sensible neuronopathie, demyelinisations etc.). Current aspects of the diagnosis, prognosis and therapeutic considerations of the management of these complications of Sjögren’s syndrome are addressed by this review. While treatment options of most of these complications are limited and lack robust scientific evidence, management of patients with this disorder require an interdisciplinary approach.

Schlüsselwörter

Sjögren-Syndrom - Autoimmunität - Lymphome - Thrombose - Parodontitis

Keywords

Sjögren´s syndrome - autoimmunity - lymphoma - thrombosis - parodontitis

• Literatur

• 1 Hansen A, Dorner T. Sjogren syndrome. Internist 2010; 51: 1267-1279.
• 2 Shiboski SC, Shiboski CH, Criswell LA. et al. American College of Rheumatology classification criteria for Sjogren’s syndrome: A data-driven, expert consensus approach in the Sjogren’s International Collaborative Clinical Alliance Cohort. Arthritis Care & Research 2012; 64: 475-487.
• 3 Vitali C, Bootsma H, Bowman SJ. et al. Classification criteria for Sjogren’s syndrome: we actually need to definitively resolve the long debate on the issue. Annals of the Rheumatic Diseases 2013; 72: 476-478.
• 4 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: 554-558.
• 5 Seror R, Ravaud P, Mariette X. et al. EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjogren’s syndrome. Annals of the Rheumatic Diseases 2011; 70: 968-972.
• 6 Westhoff G, Dorner T, Zink A. Fatigue and depression predict physician visits and work disability in women with primary Sjogren’s syndrome: results from a cohort study. Rheumatology 2012; 51: 262-269.
• 7 Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases - A meta-analysis. Archives of Internal Medicine 2005; 165: 2337-2344.
• 8 Leandro MJ, Isenberg DA. Rheumatic diseases and malignancy - is there an association?. Scandinavian Journal of Rheumatology 2001; 30: 185-188.
• 9 Theander E, Vasaitis L, Baecklund E. et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjogren’s syndrome. Annals of the Rheumatic Diseases 2011; 70: 1363-1368.
• 10 Vivino FB, Al-Hashimi I, Khan Z. et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjogren syndrome - A randomized, placebo-controlled, fixed-dose, multicenter trial. Archives of Internal Medicine 1999; 159: 174-181.
• 11 Gottenberg JE, Ravaud P, Puechal X. et al. Inefficacy of Hydroxychoroquine in Primary Sjogren’s Syndrome: Results of the Joquer Randomized Placebo-Controlled Trial in Primary Sjogren’s Syndrome. Arthritis and Rheumatism 2012; 64: 4170-4171.
• 12 Hoppe B, Dorner T. Coagulation and the fibrin network in rheumatic disease: a role beyond haemostasis. Nature Reviews Rheumatology 2012; 8: 738-746.
• 13 Haga HJ, Jacobsen EM, Peen E. Incidence of thromboembolic events in patients with primary Sjogren’s syndrome. Scandinavian Journal of Rheumatology 2008; 37: 127-129.
• 14 Hussein SZ, Jacobsson LTH, Lindquist PG, Theander E. Pregnancy and fetal outcome in women with primary Sjogren’s syndrome compared with women in the general population: a nested case-control study. Rheumatology 2011; 50: 1612-1617.
• 15 Hoppe B, Burmester GR, Dorner T. Heparin or aspirin or both in the treatment of recurrent abortions in women with antiphospholipid antibody (syndrome). Current Opinion in Rheumatology 2011; 23: 299-304.
• 16 Gris JC, Bouvier S, Molinari N. et al. Comparative incidence of a first thrombotic event in purely obstetric antiphospholipid syndrome with pregnancy loss: the NOH-APS observational study. Blood 2012; 119: 2624-2632.
• 17 Dorner T, Hiepe F, Goldner B, Apostoloff E. Investigations Into Ro-Specific Antibody-Associated Congenital Cardiac Conduction Defects. Clinical Investigator 1992; 70: 492-496.
• 18 Dorner T, Trebeljahr G, Goldner B. et al. Detection of Autoantibodies to Ro(Ss-A), La(Ss-B) and U1Rnp in Different Congenital Heart Rhythm Disorders Using Immunoblot and Enzyme-Immunoassay. Journal of Autoimmunity 1994; 7: 93-106.
• 19 Dorner T, Chaoui R, Feist E. et al. Significantly Increased Maternal and Fetal Igg Autoantibody Levels to 52 Kd Ro(Ss-A) and La(Ss-B) in Complete Congenital Heart-Block. Journal of Autoimmunity 1995; 8: 675-684.
• 20 Feist E, Chaoui R, Goldner R. et al. Neonatal lupus erythematosus and complete congenital heart block. Aktuelle Rheumatologie 2003; 28: 102-107.
• 21 Feist E, Keitzer R, Gerhold K. et al. Development of systemic lupus erythematosus in a patient with congenital heart block. Arthritis and Rheumatism 2003; 48: 2697-2698.
• 22 Trucco SM, Jaeggi E, Cuneo B. et al. Use of Intravenous Gamma Globulin and Corticosteroids in the Treatment of Maternal Autoantibody-Mediated Cardiomyopathy. Journal of the American College of Cardiology 2011; 57: 715-723.
• 23 Gladman G, Silverman ED, Yuk L. et al. Fetal echo cardiographic screening of pregnancies of mothers with anti-Ro and/or anti-La antibodies. American Journal of Perinatology 2002; 19: 73-79.
• 24 Harms L, Hiepe F. Neurological manifestations in connective tissue disease. Zeitschrift fur Rheumatologie 2012; 71: 564-571.
• 25 Delalande S, de Seze J, Fauchais AL. et al. Neurologic manifestations in primary Sjogren syndrome - A study of 82 patients. Medicine 2004; 83: 280-291.
• 26 Wahren-Herlenius M, Dörner T. Immunopathogenic mechanisms of systemic autoimmune diseases. Lancet 2013; 382 (9894): 819-831.
• 27 Westhoff G, Dörner T, Cobarrubias K. et al. Dental Implants are a treatment option in patients with Sjögren’s syndrome. Ann Rheum Dis 2012; 71 (Suppl. 03) 547.