Skip to main content

Advertisement

SpringerLink
Log in

Fatal Kikuchi–Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Kikuchi–Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD can frequently mimic various diseases including infection, malignancy, and autoimmune disease. KFD has also been infrequently reported in association with SLE, which can be diagnosed previously, simultaneously, or after the diagnosis of SLE. The author presents here the first case of fatal KFD simultaneously occurred with SLE complicated with hemophagocytic syndrome and severe infection. The recognition of this association is of significance for management of affected patients with early intensive immunosuppressive therapy for favorable outcome.

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

Fig. 1

Similar content being viewed by others

References

  1. Kucukardali Y, Somazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007) Kikuchi–Fujimoto disease: analysis of 244 cases. Clin Rheumatol 26:50–54

    Article  PubMed  Google Scholar 

  2. Mahajan T, Merriman RC, Stone MJ (2007) Kikuchi–Fujimoto disease (histiocytic necrotizing lymphadentitis): report of a case with other autoimmune manifestations. Proc (Bayl Univ Med Cent) 20:149–151

    Google Scholar 

  3. Santana A, Lessa B, Galrao L, Lima I, Santiago M (2005) Kikuchi–Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 24:60–63

    Article  PubMed  Google Scholar 

  4. Quintas-Cardama A, Fraga M, Cozzi SN, Caparrini A, Maceiras F, Forteza J (2003) Fatal Kikuchi–Fujimoto disease: the lupus connection. Ann Hematol 82:186–188

    Article  PubMed  CAS  Google Scholar 

  5. Bosch X, Guilabert A, Miquel R, Campo E (2004) Enigmatic Kikuchi–Fujimoto disease: a comprehensive review. Am J Clin Pathol 122:141–152

    Article  PubMed  Google Scholar 

  6. Bosch X, Guilabert A (2006) Kikuchi–Fujimoto disease. Orphanet J Rare Dis 1:18

    Article  PubMed  Google Scholar 

  7. Larroche C, Mouthon L (2004) Pathogenesis of hemophagocytic syndrome (HPS). Autoimmune Rev 3:69–75

    Article  CAS  Google Scholar 

  8. Romanou V, Hatzinikolaou P, Mavragani KI, Meletis J, Vaiopoulos G (2006) Lupus erythematosus complicated by hemophagocytic syndrome. J Clin Rheumatol 12:301–303

    Article  PubMed  Google Scholar 

  9. Qian J, Yang CD (2007) Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review. Clin Rheumatol 26:807–810

    Article  PubMed  Google Scholar 

Download references

Disclosures

None.

Author information

Authors and Affiliations

  1. Division of Allergy and Clinical Immunology, Department of Internal Medicine, Faculty of Medicine, Chulalongkorn University Hospital, Rama IV Road, Pathumwan, Bangkok, 10330, Thailand

    Thatchai Kampitak

Authors
  1. Thatchai Kampitak
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Thatchai Kampitak.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kampitak, T. Fatal Kikuchi–Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report. Clin Rheumatol 27, 1073–1075 (2008). https://doi.org/10.1007/s10067-008-0902-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-008-0902-4

Keywords

Search

Navigation