Dr. Kumakura has received speaking fees from Dainippon Sumitomo Pharma (less than $10,000).
Autoimmune-Associated Hemophagocytic Syndrome
Clinical Characteristics and Treatment Outcomes of Autoimmune-Associated Hemophagocytic Syndrome in Adults
Article first published online: 28 JUL 2014
© 2014 The Authors. Arthritis & Rheumatology is published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.
This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
Arthritis & Rheumatology
Volume 66, Issue 8, pages 2297–2307, August 2014
How to Cite
Kumakura, S. and Murakawa, Y. (2014), Clinical Characteristics and Treatment Outcomes of Autoimmune-Associated Hemophagocytic Syndrome in Adults. Arthritis & Rheumatology, 66: 2297–2307. doi: 10.1002/art.38672
- Issue published online: 28 JUL 2014
- Article first published online: 28 JUL 2014
- Accepted manuscript online: 22 APR 2014 12:00AM EST
- Manuscript Accepted: 11 APR 2014
- Manuscript Received: 22 JUN 2013
To better define the clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome (AAHS) in adults.
Adults with AAHS (defined as pathologic evidence of hemophagocytosis without any obvious cause other than an autoimmune disease) were identified through a review of the literature.
Among 116 patients identified, underlying diseases included systemic lupus erythematosus (SLE) in 52.3%, adult-onset Still's disease (AOSD) in 26.7%, and dermatomyositis in 6.9%. Fever, lymphadenopathy, hepatomegaly, and splenomegaly were found in 86.8%, 41.0%, 41.8%, and 45.5% of patients, respectively. Cytopenia, liver dysfunction, and hyperferritinemia developed frequently, and coagulopathy was seen in 50.6% of patients. Normal or low C-reactive protein levels were characteristic of patients with underlying SLE. The most commonly used therapy was corticosteroids, which were initially administered in 95.7% of patients, with 57.7% responding. Patients with corticosteroid-refractory disease were usually treated with cyclosporine, intravenous cyclophosphamide (IV CYC), or intravenous immunoglobulin (IVIG), with IV CYC being highly effective. Treatment with biologic agents resulted in favorable effects in the majority of patients. The mortality rate was 12.9%. Male sex (odds ratio [OR] 6.47, 95% confidence interval [95% CI] 2.06–30.39, P < 0.01), dermatomyositis (OR 5.57, 95% CI 1.08–28.65, P < 0.05), and anemia (hemoglobin <8 gm/dl; OR 3.74, 95% CI 1.02–13.8, P < 0.05) were identified as factors associated with mortality.
AAHS is potentially fatal. Corticosteroids are a mainstay of initial treatment. For corticosteroid-refractory disease, IV CYC may be beneficial as compared with cyclosporine or IVIG. Treatment that proceeds directly from corticosteroids to biologic agents is promising.