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Rheumatology Advance Access originally published online on September 9, 2008
Rheumatology 2008 47(11):1686-1691; doi:10.1093/rheumatology/ken342
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases

S. Fukaya1, S. Yasuda1, T. Hashimoto1, K. Oku1, H. Kataoka1, T. Horita1, T. Atsumi1 and T. Koike1

1Department of Medicine II, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Correspondence to: S. Yasuda, Department of Medicine II, Hokkaido University Graduate School of Medicine, N15 W7, Kita-ku, Sapporo 060-8638, Japan. E-mail: syasuda{at}med.hokudai.ac.jp


  Abstract

Objectives. Haemophagocytic syndrome (HPS) is known as a relatively rare complication in autoimmune diseases. Here we analysed the clinical features of HPS in patients with systemic autoimmune diseases.

Methods. One thousand and fourteen patients with systemic autoimmune diseases admitted to Hokkaido University Hospital from 1997 to 2007 were recruited [350 SLE, 136 RA, 98 polymyositis/dermatomyositis (PM/DM), 88 SSc, 91 vasculitis syndrome, 37 primary SS, 26 adult onset Still's disease (AOSD) and 188 other diseases]. Clinical features and treatment outcomes were retrospectively analysed.

Results. Thirty cases (3.0%) fulfilled HPS criteria (progressive cytopenia in two or more lineages and haemophagocytosis in reticuloendothelial systems). Underlying diseases were SLE (18), RA (2), PM/DM (2), SSc (2), vasculitis (1), SS (2) and AOSD (3). Nineteen patients were diagnosed as having autoimmune-associated HPS, eight infection-associated, one drug-induced and one developed HPS after haematopoietic stem cell transplantation. For the treatment of HPS, high-dose corticosteroid monotherapy was given in 26 cases, being effective in 12 (46%). Ten out of 15 patients with corticosteroid-resistant autoimmune-associated HPS were treated with CsA, cyclophosphamide or tacrolimus, leading to the remission in 80%. The overall mortality rate was 20%. Multivariate analysis showed that the presence of infections and CRP level >50 mg/l on HPS related with poor prognosis.

Conclusions. The prevalence of HPS among in-hospital patients with systemic autoimmunity is not ignorable. Administration of immunosuppressants was effective in cases with autoimmune-associated HPS, whereas prognosis was poor in infection-associated HPS.

KEY WORDS: Haemophagocytosis, Haemophagocytic syndrome, Autoimmunity, Systemic lupus erythematosus, Mortality factors

Submitted 19 May 2008; revised version accepted 16 July 2008.
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