Skip to main content
Download PDF

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Autoimmunity Highlights volume 1, pages 53–59 (2010)Cite this article

Abstract

Purpose

The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications.

Methods

The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature.

Results

The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease).

Conclusion

The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

References

  1. Ohta A, Yamaguchi M, Kaneoka H et al (1987) Adult Still’s disease: review of 228 cases from the literature. J Rheumatol 14:1139–1146

    PubMed  CAS  Google Scholar 

  2. Ohta A, Yamaguchi M, Tsunematsu T et al (1990) Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 17:1058–1063

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  PubMed Central  Article  Google Scholar 

  4. Kàdàr J, Petrovicz E (2004) Adult-onset Still’s disease. Best Practice & Research Clinical Rheumatology 18:663–676

    Article  Google Scholar 

  5. Chen DY, Lan JL, Lin FJ et al (2004) Predominance of Th1 cytokine in peripheral blood and pathological tissues of patients with active untreated adult onset Still’s disease. Ann Rheum Dis 63:1300–1306

    PubMed  CAS  PubMed Central  Article  Google Scholar 

  6. Bombardieri M, McInnes IB, Pitzalis C (2007) Interleukin-18 potential therapeutic target in chronic autoimmune/inflammatory conditions. Expert Opin Biol Ther 7:31–40

    PubMed  CAS  Article  Google Scholar 

  7. Chen DY, Lan JL, Lin FJ et al (2004) Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still’s disease. J Rheumatol 31:2189–2198

    PubMed  CAS  Google Scholar 

  8. Kawaguchi Y, Terajima H, Harigai M et al (2001) Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still’s disease. Arthritis Rheum 44:1716–1718

    PubMed  CAS  Article  Google Scholar 

  9. Kawashima M, Yamamura M, Taniai M et al (2001) Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still’s disease. Arthritis Rheum 44:550–560

    PubMed  CAS  Article  Google Scholar 

  10. Conigliaro P, Priori R, Bombardieri M et al (2009) Lymph node IL-18 expression in adult onset Still’s disease. Ann Rheum Dis 68:442–443

    PubMed  CAS  Article  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  12. Kontzias A, Efthimiou P (2008) Adult-onset Still’s disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 68:319–337

    PubMed  CAS  Article  Google Scholar 

  13. Fautrel B (2008) Adult-onset Still disease. Best Pract Res Clin Rheumatol 22:773–792

    PubMed  Article  Google Scholar 

  14. Pouchot J, Sampalis JS, Beaudet F et al (1991) Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70:118–36

    CAS  Article  Google Scholar 

  15. Singh S, Samant R, Joshi VR (2008) Adult onset Still’s disease: a study of 14 cases. Clin Rheumatol 27:35–39

    PubMed  CAS  Article  Google Scholar 

  16. Reginato AJ, Schumacher HR Jr, Baker DG et al (1987) Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 17(1:39–57

    PubMed  CAS  Article  Google Scholar 

  17. Zeng T, Zou YQ, Wu MF et al (2009) Clinical features and prognosis of adult onset Still’s disease: 61 cases from China. J Rheumatol 36:1026–31

    PubMed  CAS  Article  Google Scholar 

  18. Zhu G, Liu G, Liu Y et al (2009) Liver abnormalities in adult onset Still’s disease: a retrospective study of 77 Chinese patients. J Clin Rheumatol 15:284–288

    PubMed  Article  Google Scholar 

  19. Hong YH, Lee CK (2008) A case of adult onset Still’s disease with systemic inflammatory response syndrome complicated by fatal status epilepticus. Rheumatol Int 28:931–933

    PubMed  Article  Google Scholar 

  20. Colina M, Govoni M, Trotta F (2009) Fatal myocarditis in adultonset Still’s disease with diffuse intravascular coagulation. Rheumatol Int 29:1355–7

    PubMed  Article  Google Scholar 

  21. Gianella S, Schaer DJ, Schwarz U et al (2008) Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adultonset Still’s disease and concurrent macrophage activation syndrome. Am J Hematol 83:424–427

    PubMed  Article  Google Scholar 

  22. Arlet JB, Le TH, Marinho A et al (2006) Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis 65:1596–1601

    PubMed  PubMed Central  Article  Google Scholar 

  23. Dino O, Provenzano G, Giannuoli G et al (1996) Fulminant hepatic failure in adult onset Still’s disease. J Rheumatol 23:784–785

    PubMed  CAS  Google Scholar 

  24. Thabah MM, Singh KK, Madhavan SM et al (2008) Adult onset Still’s disease as a cause of acute liver failure. Trop Gastroenterol 29:35–36

    PubMed  Google Scholar 

  25. Hirohata S, Kamoshita H, Taketani T et al (1986) Adult Still’s disease complicated with adult respiratory distress. Arch Intern Med 146:2409–2410

    PubMed  CAS  Article  Google Scholar 

  26. Stoica GS, Cohen RI, Rossof LG (2002) Adult Still’s disease and respiratory failure in a 74 year old woman. Postgrad Med J 78:97–98

    PubMed  CAS  PubMed Central  Article  Google Scholar 

  27. Mejjad O, Vittecoq O, Tamion F et al (2001) A shock associated with adult onset Still’s disease. Joint Bone Spine 68:76–78

    PubMed  CAS  Article  Google Scholar 

  28. Franchini S, Dagna L, Salvo F, Aiello P, Baldissera E, Sabbadini MG (2010) Adult onset Still’s disease: clinical presentation in a large cohort of italian patients. 28:41–48

    CAS  Google Scholar 

  29. Priori R, Ceccarelli F, Barone F et al (2008) Clinical, biological and sonographic response to IL-1 blockade in adult onset Still’s disease. Clin Exp Rheumatol 26:933–937

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Article  Google Scholar 

  31. 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

    PubMed  CAS  PubMed Central  Article  Google Scholar 

  32. Louthrenoo W, Aramsareewong T, Sukitawut W (2001) Adult onset Still’s disease: clinical features and outcome in 16 Thai patients. J Clin Rheumatol 7:301–307

    PubMed  CAS  Article  Google Scholar 

  33. Uppal SS, Pande IR, Kumar A et al (1995) Adult onset Still’s disease in northern India: comparison with juvenile onset Still’s disease. Br J Rheumatol 34:429–34

    PubMed  CAS  Article  Google Scholar 

  34. Uppal SS, Al-Mutairi M, Hayat S et al (2007) Ten years of clinical experience with adult onset Still’s disease: is the outcome improving? Clin Rheumatol 26:1055–1060

    PubMed  Article  Google Scholar 

  35. Masson C, Le loet X, Liote F et al (1995) Adult Still’s disease: part I. Manifestations and complications in sixty-five cases in France. Rev Rhum Engl Ed 62:748–757

    PubMed  CAS  Google Scholar 

  36. Andres E, Kurtz JE, Perrin AE et al (2003) Retrospective monocentric study of 17 patients with adult Still’s disease, with special focus on liver abnormalities. Hepatogastroenterology 50:192–195

    PubMed  Google Scholar 

  37. McFarlane M, Harth M, Wall WJ (1997) Liver transplantation in adult Still’s disease. J Rheum 24:2038–2041

    PubMed  CAS  Google Scholar 

  38. Ott SJ, Baron A, Berghaus T et al (2003) Liver failure in adult Still’s disease during corticosteroid treatment. Eur J Gastroenterol Hepatol 15:87–90

    PubMed  Article  Google Scholar 

  39. Jung JH, Jun JB, Yoo DH et al (2000) High toxicity of sulfasalazine in adult-onset Still’s disease. Clin Exp Rheumatol 18:245–248

    PubMed  CAS  Google Scholar 

  40. Crowley J, Situnayake RD (1992) Sulphasalazine induced hepatitis in adult Still’s disease. Ann Rheum Dis 51:1264–1265

    PubMed  CAS  PubMed Central  Article  Google Scholar 

  41. Janssen HL, van Laar JM, van Hoek B et al (1999) Severe hepatitis and pure red cell aplasia in adult Still’s disease: good response to immunosuppressive therapy. Dig Dis Sci 44:1639–1642

    PubMed  CAS  Article  Google Scholar 

  42. Wouters JM, van de Putte LB (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 

  43. Mehrpoor G, Owlia MB, Soleimani H et al (2008) Adult onset Still’s disease: a report of 28 cases and review of the literature. Mod Rheumatol 18:480–485

    PubMed  Article  Google Scholar 

  44. Catagay Y, Gul A, Catagay A et al (2009) Adult-onset Still’s disease. Intern J Clin Pract 63:1050–1055

    Article  Google Scholar 

  45. Ames PR, Walker E, Aw D et al (2009) Multi-organ failure in adult onset Still’s disease: a septic disguise. Clin Rheumatol 28(Suppl 1):S3–S6

    PubMed  Article  Google Scholar 

  46. Krzossok S, Benck U, Van Der Woude FJ et al (2004) Disseminated intravascular coagulation, perimyocarditis and empyema in adult onset Still’s disease. Dtsch Med Wochenschr 129:2535–2537

    PubMed  CAS  Article  Google Scholar 

  47. Aray Y, Handa T, Mitani K (2004) Adult onset Still’s disease presenting with disseminated intravascular coagulation. Rinsho Ketsueki 45:316–318

    Google Scholar 

  48. Park JH, Bae JH, Choi YS et al (2004) Adult-onset Still’s disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. J Korean Med Shi 19:137–141

    Article  Google Scholar 

  49. Yokoyama M, Suwa A, Shinozawa T et al (1995) A case of adult onset Still’s disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation. Nihon Rinsho Meneki Gakkai Kaishi 18:207–214

    PubMed  CAS  Article  Google Scholar 

  50. Iglesias J, Sathiraju S, Marik PE (1999) Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still’s disease: clinical response with high dose pulse methylprednisolone therapy. Chest 115:1738–1740

    PubMed  CAS  Article  Google Scholar 

  51. Takeshita A, Takeuchi T, Nakagawa A et al (2000) Adult onset Still’s disease with hemophagocytic syndrome and severe liver dysfunction. Hepatol Res 17:139–144

    PubMed  Article  Google Scholar 

  52. Félix FH, Leal LK, Fontenele JB (2009) Cloak and dagger: the case for adult onset Still disease and hemophagocytic lymphohistiocytosis. Rheumatol Int 29:973–974

    PubMed  Article  Google Scholar 

  53. Cavallasca JA, Vigliano CA, Perandones CE et al (2009) Myocarditis as a form of relapse in two patients with adult Still’s disease. Rheumatol Int. DOI: 10.1007/s00296-009-1031-3

  54. Vandergheinst F, Gosset J, Van de Borne P et al (2005) Myopericarditis revealing adult onset Still’s disease. Acta Clin Belg 60:205–208

    Article  Google Scholar 

  55. Nishimagi E, Hirata S, Kawaguchi Y et al (2004) Myocardial dysfunction in a patient with adult onset Still’s disease. Clin Exp Rheumatol 22:506–507

    PubMed  CAS  Google Scholar 

  56. Hosaka S, Takashina N, Ishikawa A et al (1992) Adult Still’s disease with myocarditis and peritonitis. Intern Med 31:812–815

    PubMed  CAS  Article  Google Scholar 

  57. Kuek A, Weerakoon A, Ahmed K et al (2007) Adult-onset Still’s disease and myocarditis: successful treatment with intravenous immunoglobulin and maintenance of remission with etanercept. Rheumatology (Oxford) 46:1043–1044

    CAS  Article  Google Scholar 

  58. Prieur AM (1996) Intravenous immunoglobulins in Still’s disease: still controversial, still unproven. J Rheumatol 23:797–800

    PubMed  CAS  Google Scholar 

  59. Perez MG, Rodwing FR Jr (2003) Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still’s disease. South Med J 96:46–49

    PubMed  Article  Google Scholar 

  60. Diamond JR (1997) Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura complicating adult Still’s Disease: remission induced with intravenous immunoglobulin g. J Nephrol 10:253–257

    PubMed  CAS  Google Scholar 

  61. Denault A, Dimopoulos MA, Fitzcharles MA (1990) Meningoencephalitis and peripheral neuropathy complicating adult Still’s disease. J Rheumatol 17:698–700

    PubMed  CAS  Google Scholar 

  62. Markusse HM, Stolk B, Van der Mey AG et al (1988) Sensorineural hearing loss in adult Still’s disease. Ann Rheum Dis 47:600–602

    PubMed  CAS  PubMed Central  Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161, Rome, Italy

    Roberta Priori, Serena Colafrancesco, Angelica Gattamelata, Manuela Di Franco, Ugo Di Tondo & Guido Valesini

Authors
  1. Roberta Priori
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Serena Colafrancesco
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Angelica Gattamelata
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Manuela Di Franco
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ugo Di Tondo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Guido Valesini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Roberta Priori.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Priori, R., Colafrancesco, S., Gattamelata, A. et al. Adult-onset Still disease: a rare disorder with a potentially fatal outcome. Autoimmun Highlights 1, 53–59 (2010). https://doi.org/10.1007/s13317-010-0009-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13317-010-0009-1

Keywords

  • Adult-onset Still disease
  • Fever
  • Auto inflammatory diseases
  • Disseminated intravascular coagulation
  • Myocarditis

Autoimmunity Highlights

ISSN: 2038-3274

Contact us