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

Rheumatology (Oxford). 2008 Nov;47(11):1686-91. doi: 10.1093/rheumatology/ken342. Epub 2008 Sep 9.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / mortality
  • Bacterial Infections / complications
  • Bacterial Infections / drug therapy
  • Bacterial Infections / mortality
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lymphohistiocytosis, Hemophagocytic / complications*
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / mortality
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Tacrolimus / therapeutic use
  • Virus Diseases / complications
  • Virus Diseases / drug therapy
  • Virus Diseases / mortality
  • Young Adult


  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclosporine
  • Cyclophosphamide
  • Tacrolimus