RSS-Feed abonnieren
DOI: 10.1055/s-0031-1281572
© Georg Thieme Verlag KG Stuttgart · New York
Adulter Morbus Still
Adult-onset Still’s diseasePublikationsverlauf
                     eingereicht: 21.6.2011
                     
                     akzeptiert: 1.8.2011
                     
Publikationsdatum:
10. August 2011 (online)

Zusammenfassung
Der adulte Morbus Still ist eine seltene entzündliche Systemerkrankung. Leitsymptome/-manifestationen sind Fieber, Arthralgien oder Arthritis, Myalgien, ein typischer Hautausschlag, Halsschmerzen, Hepatosplenomegalie, Lymphadenopathie und Serositis. Zahlreiche weitere Symptome und Organmanifestationen sind jedoch möglich. Das klinische Erscheinungsbild ist variabel und kann leicht bis lebensbedrohlich ausgeprägt sein. Der Erkrankungsverlauf ist spontan-limitierend, intermittierend oder chronisch. Da ein beweisendes diagnostisches Testverfahren nicht vorhanden ist, kann die Diagnose erst nach Ausschluss zahlreicher, vor allem infektiöser, maligner und autoimmuner Differentialdiagnosen gestellt werden. Zur Therapie können nichtsteroidale Antiphlogistika, Glucocorticoide, Basistherapeutika und Biologika eingesetzt werden.
Abstract
Adult-onset Still’s disease is a rare inflammatory systemic disease. Cardinal symptoms/manifestations are fever, arthralgias or arthritis, myalgias, the typical skin rash, sore throat, hepatosplenomegaly, lymphadenopathy and serositis. Several other symptoms and organ involvements are possible. The clinical picture is variable with mild to life-threatening courses. The disease is self-limiting, intermittently active or chronic. Because of the lack of a defined diagnostic test the diagnosis of AOSD can only be made after exclusion of several differential diagnoses in particular of infectious, malignant and autoimmune origin. For therapy non-steroidal anti-inflammatory drugs, glucocorticoids, disease modifying antirheumatic drugs and biologics can be used.
Schlüsselwörter
adulter Morbus Still - Morbus Still des Erwachsenen - adult-onset Still’s disease (AOSD)
Keywords
Still’s disease - adult-onset Still’s disease - AOSD
Literatur
- 1 
            Ahmadi-Simab K, Lamprecht P, Jankowiak C, Gross W L. 
            Successful treatment
            of refractory adult onset Still’s disease with rituximab. 
            Ann Rheum Dis. 
            2006; 
            65 
            1117-1118 
            
            Reference Ris Wihthout Link
- 2 
            Bagnari V, Colina M, Ciancio G, Govoni M, Trotta F. 
            Adult-onset Still’s disease. 
            Rheumatol Int. 
            2010; 
            30 
            855-862 
            
            Reference Ris Wihthout Link
- 3 
            Benucci M, Li G F, Del Rosso A, Manfredi M. 
            Adalimumab (anti-TNF-alpha)
            therapy to improve the clinical course of adult-onset Still’s
            disease: the first case report. 
            Clin Exp Rheumatol. 
            2005; 
            23 
            733 
            
            Reference Ris Wihthout Link
- 4 
            Bisagni-Faure A, Job-Deslandre C, Menkes C J. 
            Intravenous methylprednisolone pulse therapy
            in Still’s disease. 
            J Rheumatol. 
            1992; 
            19 
            1487-1488 
            
            Reference Ris Wihthout Link
- 5 
            Bywaters E GL. 
            Still’s disease in the adult. 
            Ann Rheum Dis. 
            1971; 
            30 
            121-133 
            
            Reference Ris Wihthout Link
- 6 
            Dechant C, Schauenberg P, Antoni C E, Kraetsch H G, Kalden J R, Manger B. 
            Langzeitergebnisse
            einer TNF-Blockade beim Morbus Still im Erwachsenenalter. 
            Dtsch
            Med Wochenschr. 
            2004; 
            129 
            1308-1312 
            
            Reference Ris Wihthout Link
- 7 
            Dechant C, Schulze-Koops H. 
            Adult-Onset Still’s
            Disease. 
            Akt Rheumatol. 
            2008; 
            33 
            108-114 
            
            Reference Ris Wihthout Link
- 8 
            Efthimiou P, Paik P K, Bielory L. 
            Diagnosis and management of adult onset Still’s disease. 
            Ann Rheum Dis. 
            2006; 
            65 
            564-572 
            
            Reference Ris Wihthout Link
- 9 
            Efthimiou P, Georgy S. 
            Pathogenesis and management
            of adult-onset Still`s Disease. 
            Semin Arthritis
            Rheum. 
            2006; 
            36 
            144-152 
            
            Reference Ris Wihthout Link
- 10 
            Evensen K J, Nossent H C. 
            Epidemiology
            and outcome of adult-onset Still’s disease in Northern
            Norway. 
            Scand J Rheumatol. 
            2006; 
            35 
            48-51 
            
            Reference Ris Wihthout Link
- 11 
            Fautrel B. 
            Adult-onset Still disease. 
            Best Pract Res Clin Rheumatol. 
            2008; 
            22 
            773-792 
            
            Reference Ris Wihthout Link
- 12 
            Fautrel B, Sibilia J, Mariette X, Combe B. 
            Tumour necrosis factor
            alpha blocking agents in refractory adult Still’s disease:
            an observational study of 20 cases. 
            Ann Rheum Dis. 
            2005; 
            64 
            262-266 
            
            Reference Ris Wihthout Link
- 13 
            Fautrel B, Zing E, Golmard J L
            P. et al .
            Proposal for a new set of classification
            criteria for adult-onset still disease. 
            Medicine (Baltimore). 
            2002; 
            81 
            194-200 
            
            Reference Ris Wihthout Link
- 14 
            Fautrel B, Le Moël G, Saint-Marcoux B. et al .
            Diagnostic value of ferritin and
            glycosylated ferritin in adult onset Still’s disease. 
            J Rheumatol. 
            2001; 
            28 
            322-329 
            
            Reference Ris Wihthout Link
- 15 
            Hoshino T, Ohta A, Yang D. et
            al .
            Elevated serum interleukin 6, interferon-gamma,
            and tumor necrosis factos-alpha levels in patients with adult Still’s
            disease. 
            J Rheumatol. 
            1998; 
            25 
            396-398 
            
            Reference Ris Wihthout Link
- 16 
            Hot A, Toh M L, Coppéré B. 
            Reactive hemophagocytic syndrome in adult-onset
            Still disease: clinical features and long-term outcome: a case-control
            study of 8 patients. 
            Medicine (Baltimore). 
            2010; 
            89 
            37-46 
            
            Reference Ris Wihthout Link
- 17 
            Husni M E, Maier A L, Mease P J. et al .
            Etanercept in the treatment
            of adult patients with Still’s disease. 
            Arthritis
            Rheum. 
            2002; 
            46 
            1171-1176 
            
            Reference Ris Wihthout Link
- 18 
            Jiang L, Wang Z, Dai X, Jin X. 
            Adult-onset Still’s disease in
            a Chinese population. 
            J Rheumatol. 
            2011; 
            38 
            741-746 
            
            Reference Ris Wihthout Link
- 19 
            Jung J H, Jun J B, Yoo D H. et al .
            High toxicity of sulfasalazine in adult-onset
            Still’s disease. 
            Clin Exp Rheumatol. 
            2000; 
            18 
            245-248 
            
            Reference Ris Wihthout Link
- 20 
            Kawashima M, Yamamura M, Taniai M. et al .
            Levels of interleukin-18 and its binding
            inhibitors in the blood circulation of patients with adult-onset
            Still’s disease. 
            Arthritis Rheum. 
            2001; 
            44 
            550-560 
            
            Reference Ris Wihthout Link
- 21 
            Khraishi M, Fam A G. 
            Treatment of fulminant
            adult Still’s disease with intravenous pulse methylprednisolone
            therapy. 
            J Rheumatol. 
            1991; 
            18 
            1088-1090 
            
            Reference Ris Wihthout Link
- 22 
            Kötter I, Wacker A, Koch S. et
            al .
            Anakinra in patients with treatment-resistant adult-onset
            Still’s disease: four case reports with serial cytokine
            measurements and a review of the literature. 
            Semin Arthritis Rheum. 
            2007; 
            37 
            189-197 
            
            Reference Ris Wihthout Link
- 23 
            Kötter I, Schedel J, Kümmerle-Deschner J B. 
            Periodische Fiebersyndrome / autoinflammatorische
            Syndrome. 
            Z Rheumatol. 
            2009; 
            68 
            137-148 
            
            Reference Ris Wihthout Link
- 24 
            Laskari K, Tzioufas A G, Moutsopoulos H M. 
            Efficacy and long-term follow-up
            of IL-1R inhibitor anakinra in adults with Still’s disease:
            a case-series study. 
            Arthritis Res Ther. 
            2011; 
            13 
            R91 
            
            Reference Ris Wihthout Link
- 25 
            Lequerré T, Quartier P, Rosellini D. et al .
            Interleukin-1 receptor antagonist (anakinra)
            treatment in patients with systemic-onset juvenile idiopathic arthritis
            or adult onset Still disease: preliminary experience in France. 
            Ann Rheum Dis. 
            2008; 
            67 
            302-308 
            
            Reference Ris Wihthout Link
- 26 
            Manger B. 
            Adult onset Still’s disease. 
            Z Rheumatol. 
            2008; 
            67 
            415-422 
            
            Reference Ris Wihthout Link
- 27 
            Manger B, Rech J, Schett G. 
            Use of methotrexate in adult-onset Still’s disease. 
            Clin Exp Rheumatol. 
            2010; 
            28 
            (5 Suppl 61) 
            S168-171 
            
            Reference Ris Wihthout Link
- 28 
            Masson C, Le Loët X, Lioté F. et al .
            Comparative study of 6 types
            of criteria in adult Still’s disease. 
            J Rheumatol. 
            1996; 
            62 
            495-497 
            
            Reference Ris Wihthout Link
- 29 
            Masson C, Le Loët X, Lioté F. et al .
            Adult Still’s disease.
            Part II. Management, outcome, and prognostic factors. 
            Rhev
            Rum Engl Ed. 
            1995; 
            62 
            758-765 
            
            Reference Ris Wihthout Link
- 30 
            Moulis G, Sailler L, Astudillo L, Pugnet G, Arlet P. 
            May anakinra be used earlier in adult onset Still disease?. 
            Clin Rheumatol. 
            2010; 
            29 
            1199-1200 
            
            Reference Ris Wihthout Link
- 31 
            Naumann L, Feist E, Natusch A. et al .
            IL1-receptor antagonist anakinra provides
            long-lasting efficacy in the treatment of refractory adult-onset
            Still’s disease. 
            Ann Rheum Dis. 
            2001; 
            69 
            466-467 
            
            Reference Ris Wihthout Link
- 32 
            Ohta A, Yamaguchi M, Tsunematsu T. et al .
            Adult Still’s disease: a multicenter
            survey of Japanese patients. 
            J Rheumatol. 
            1990; 
            17 
            1058-163 
            
            Reference Ris Wihthout Link
- 33 
            Perdan-Pirkmajer K, Praprotnik S, Tomi M. 
            A case of refractory adult-onset Still’s disease successfully
            controlled with tocilizumab and a review of the literature. 
            Clin
            Rheumatol. 
            201; 
            29 
            1465-1467 
            
            Reference Ris Wihthout Link
- 34 
            Pouchot J, Sampalis J S, Beaudet F. et al .
            Adult Still’s disease: manifestations,
            disease course, and outcome in 62 patients. 
            Medicine. 
            1991; 
            70 
            118-136 
            
            Reference Ris Wihthout Link
- 35 
            Puéchal X, DeBandt M, Berthelot J M. et al .
            Tocilizumab in refractory adult Still’s
            disease. 
            Arthritis Care Res. 
            2011; 
            63 
            155-159 
            
            Reference Ris Wihthout Link
- 36 
            Quartuccio L, Maset M, De Vita S. 
            Efficacy of abatacept in a refractory case of adult-onset Still’s
            disease. 
            Clin Exp Rheumatol. 
            2010; 
            28 
            265-267 
            
            Reference Ris Wihthout Link
- 37 
            Reginato A J, Schumacher Jr H R, Baker D G, O’Connor C R, Ferreiros J. 
            Adult onset Still’s
            disease: experience in 23 patients and literature review with emphasis
            on organ failure. 
            Semin Arthritis Rheum. 
            1987; 
            17 
            39-57 
            
            Reference Ris Wihthout Link
- 38 
            Thonhofer R, Hiller M, Just H, Trummer M, Siegel C, Dejaco C. 
            Treatment of refractory adult-onset
            still’s disease with tocilizumab: report of two cases and
            review of the literature. 
            Rheumatol Int. 
            2011; Epub
            Jan 15; 
            
            Reference Ris Wihthout Link
- 39 
            Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T. et al .
            Preliminary
            criteria for classification of adult Still’s disease. 
            J Rheumatol. 
            1992; 
            19 
            424-430 
            
            Reference Ris Wihthout Link
Dr. med. Claudia Dechant
         Rheumaeinheit, Medizinische Poliklinik
Klinikum
         der Universität 
München – Campus
         Innenstadt
         
         Pettenkoferstr. 8a
         
         80336 München
         
         eMail: claudia.dechant@med.uni-muenchen.de
         
         
 
     
      
    