Skip to main content
SpringerLink
Log in

Adulter Morbus Still, Fieber, Diagnose und Therapie

Adult onset Still’s disease, fever, diagnosis and therapy

  • Leitthema
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Der adulte Morbus Still (engl. „adult onset Still’s disease“, AOSD) mit einer Inzidenz zwischen 1 und 3 Fällen pro 1 Mio. Einwohner gehört zu den diagnostisch aufwendigsten Fiebererkrankungen. Das Fehlen von Biomarkern und seine Ähnlichkeit mit infektiösen, malignen und rheumatischen Erkrankungen führt häufig zu einer protrahierten Diagnose. Der vorliegende Beitrag soll daher versuchen, über den Kenntnisstand der klinisch relevanten Symptome, die Laborparameter für die Diagnostik des AOSD sowie die neuen Therapiemöglichkeiten eine Übersicht zu geben.

Abstract

Adult onset Still’s disease (AOSD) with an incidence of 1–3 cases per 1 million belongs to the most difficult diagnosis of febrile diseases. The lack of biomarkers and its similarity to infectious and malignant and rheumatic diseases lead to a prolongation of its diagnosis. The following report focuses on providing an overview of the current knowledge of relevant symptoms and laboratory parameters for the diagnosis of AOSD and new treatment possibilities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Literatur

  1. Bywaters EG (1971) Still’s disease in the adult. Ann Rheum Dis 30:121–133

    Article  PubMed  CAS  Google Scholar 

  2. Magadur-Joly G, Billaud E, Barrier JH et al (1995) Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54:587–590

    Article  PubMed  CAS  Google Scholar 

  3. Wakai K, Ohta A, Tamakoshi A (1997) Estimated prevalence and incidence of adult Still’s disease: findings by a nationwide epidemiological survey in Japan. J Epidemiol 7:221–225

    Article  PubMed  CAS  Google Scholar 

  4. Wouters JM, Putte LB van de (1986) Adult-onset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med 61:1055–1065

    PubMed  CAS  Google Scholar 

  5. Colina M, Zucchini W, Ciancio G et al (2011) The evolution of adult-onset Still disease: an observational and comparative study in a cohort of 76 Italian patients. Semin Arthritis Rheum 41(2):279–285

    Article  PubMed  Google Scholar 

  6. Pouchot J, Sampalis JS, Beaudet F et al (1991) Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70:118–136

    Google Scholar 

  7. Fautrel B, Zing E, Golmard JL et al (2002) Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore) 81:194–200

    Google Scholar 

  8. Kong X, Xu D, Zhang W et al (2010) Clinical features and prognosis in adult-onset still’s disease: a study of 104 cases. Clin Rheumatol 29:1015–1019

    Article  PubMed  Google Scholar 

  9. Zenone T (2007) Fever of unknown origin in rheumatic diseases. Indect Dis Clin North Am 21:1115–1135

    Article  Google Scholar 

  10. Cunha BA (2004) Fever of unknown origin caused by adult juvenile rheumatoid arthritis: the diagnostic significance of double quotidian fevers and elevated serum ferritin levels. Heart Lung 33:417–421

    Article  PubMed  Google Scholar 

  11. Larson EB (1985) Adult Still’s disease – recognition of a clinical syndrome and recent experience. West J Med 142:665–671

    PubMed  CAS  Google Scholar 

  12. Isdale IC, Bywaters EG (1956) The rash of juvenile rheumatoid arthritis. Q J Med 3:377–387

    Google Scholar 

  13. Cush JJ, Medsger TA Jr, Christy WC et al (1987) Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum 30(2):186–194

    Article  PubMed  CAS  Google Scholar 

  14. Knockaert DC, Vanneste U, Bobbaers HJ (1993) Recurrent or episodic fever of unknown origin: review of 45 cases and survey of the literature. Medicine (Baltimore) 72:184–196

    Google Scholar 

  15. Jeon YK, Paik JH, Park SS et al (2004) Spectrum of lymph node pathology in adult onset Still’s disease; analysis of 12 patients with one follow up biopsy. J Clin Pathol 57:1052–1056

    Article  PubMed  CAS  Google Scholar 

  16. Ohta A, Matsumoto M, Ohta T (1988) Still’s disease associated with necrotizing lymphadenitis (Kikuchi’s disease): report of 3 cases. J Rheumatol 15:981–983

    PubMed  CAS  Google Scholar 

  17. Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 65:564–572

    Article  PubMed  CAS  Google Scholar 

  18. Crispin JC, Martinez-Banos D, Alcocer-Varela J (2005) Adult-onset still disease as the cause of fever of unknown origin. Medicine (Baltimore) 6:331–337

    Google Scholar 

  19. Zandman-Goddard G, Shoenfeld Y (2007) Ferritin in autoimmune diseases. Autoimmun Rev 6:457–463

    Article  PubMed  CAS  Google Scholar 

  20. Vignesa S, Le Moele G, Fautrelh B et al (2000) Percentage of glycosylated serum ferritin remains low throughout the course of adult onset Still’s disease. Ann Rheum Dis 59:347–350

    Article  Google Scholar 

  21. Konijn AM, Kaplan R, Or R, Matzner Y (1992) Glycosylated serum ferritin in patients with hematological malignancies before and after bone marrow transplantation. Leuk Lymphoma 7(1–2):151–156

    Google Scholar 

  22. Reginato AJ, Schumacher HR Jr, Baker DG (1987) Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 17:39–57

    Article  PubMed  CAS  Google Scholar 

  23. Goldman JA, Beard MR, Casey HL (1980) Acute febrile juvenile rheumatoid arthritis in adults: cause of poly-arthritis and fever. South Med J 73:555–563

    Article  PubMed  CAS  Google Scholar 

  24. Yamaguchi M, Ohta A, Tsunematsu T (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–430

    PubMed  CAS  Google Scholar 

  25. Jiang L, Wang Z, Dai X, Jin X (2011) Evaluation of clinical measures and different criteria for diagnosis of adult-onset still’s disease in a Chinese population. J Rheumatol 38:741–746

    Article  PubMed  Google Scholar 

  26. Fautrel B, Sibilia J, Mariette X (2005) Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis 64:262–266

    Article  PubMed  CAS  Google Scholar 

  27. Kraetsch HG, Antoni C, Kalden JR (2001) Successful treatment of a small cohort of patients with adult onset of Still’s disease with infliximab: first experiences. Ann Rheum Dis 60:55–57

    Article  Google Scholar 

  28. Husni ME, Maier AL, Mease PJ (2002) Etanercept in the treatment of adult patients with Still’s disease. Arthritis Rheum 46:1171–1176

    Article  PubMed  CAS  Google Scholar 

  29. Lequerre T, Quartier P, Rosellini D (2008) 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 67:302–308

    Article  PubMed  CAS  Google Scholar 

  30. Fitzgerald AA, Leclercq SA, Yan A (2005) Rapid responses to anakinra in patients with refractory adult-onset Still’s disease. Arthritis Rheum 52:1794–1803

    Article  PubMed  CAS  Google Scholar 

  31. Naumann L, Feist E, Natusch A et al (2010) IL1-receptor antagonist anakinra provides long-lasting efficacy in the treatment of refractory adult-onset Still’s disease. Ann Rheum Dis 69:466–467

    Article  PubMed  CAS  Google Scholar 

  32. Puechal X, deBandt M, Breban M et al (2011) Tocilizumab in refractory adult Still’s disease. Arthritis Care Res (Hoboken) 63:155–159

    Article  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt für sich und seinen Koautor an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Klinik für Immunologie und Rheumatologie, Medizinische Hochschule Hannover, Carl-Neuberg Str. 1, 30625, Hannover, Deutschland

    N.T. Baerlecken & R.E. Schmidt

Authors
  1. N.T. Baerlecken
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. R.E. Schmidt
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to N.T. Baerlecken.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Baerlecken, N., Schmidt, R. Adulter Morbus Still, Fieber, Diagnose und Therapie. Z. Rheumatol. 71, 174–180 (2012). https://doi.org/10.1007/s00393-011-0859-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-011-0859-6

Schlüsselwörter

Keywords

Search

Navigation