Subscribe to RSS
DOI: 10.1055/s-0031-1281572
© Georg Thieme Verlag KG Stuttgart · New York
Adulter Morbus Still
Adult-onset Still’s diseasePublication History
eingereicht: 21.6.2011
akzeptiert: 1.8.2011
Publication Date:
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