Predictors of the response to treatment in acute lupus hemophagocytic syndrome.
OBJECTIVE
The objective of this paper is to identify predictors for the response to treatment of acute lupus hemophagocytic syndrome (ALHS).


METHODS
We reviewed seven cases with ALHS admitted to our hospital and published ALHS cases identified in the 2001-2014 Medline database, and then conducted univariate and multivariate analyses to identify predictors for the response to treatment.


RESULTS
Review of our cases showed a significant and negative correlation between serum ferritin and anti-DNA antibody (p = 0.0025). All three patients treated with cyclosporine A (CsA) were considered responders despite high serum ferritin and corticosteroid resistance. We also reviewed 93 patients with ALHS identified in 46 articles. Multiple logistic regression analysis identified C-reactive protein (CRP) (OR 0.83, p = 0.042) and hemoglobin (OR 1.53, p = 0.026) measured at diagnosis of ALHS as significant predictors of the response to corticosteroid monotherapy. Moreover, among 32 patients treated with CsA, serum ferritin was significantly higher in CsA responders (12163 ± 16864 µg/l, n = 22) than in non-responders (3456 ± 6267/µg/l, p = 0.020, n = 10). Leukocyte count was significantly lower in the CsA responders (1940.0 ± 972.3/µl) than in the non-responders (3253 ± 2198/µl, p = 0.034).


CONCLUSIONS
Low CRP and high hemoglobin can predict a positive response to corticosteroid monotherapy while high serum ferritin and low leukocyte count can predict a positive response to CsA in patients with ALHS and therefore, when corticosteroid monotherapy is not effective in such cases, CsA could be the first choice of an additional immunosuppressive agent.
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Takahashi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Tsuboi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Kurata
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Takahashi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Inoue
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Ebe
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Yokosawa
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Hagiwara
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Hirota
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Asashima
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Kaneko
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Kawaguchi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Kurashima
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Miki
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Umeda
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Y
Kondo
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Ogishima
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Suzuki
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
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Matsumoto
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan tsumida@md.tsukuba.ac.jp.
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Sumida
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D002097:C-Reactive Protein / Q000378:metabolism; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood* / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D012189:Retrospective Studies; D055815:Young Adult
D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D006454:Hemoglobins; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D002097:C-Reactive Protein; D005293:Ferritins; D011239:Prednisolone
Response to therapy; acute lupus hemophagocytic syndrome; corticosteroids; cyclosporine A