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 (1216316864 mu g/l, n=22) than in non-responders (3456 +/- 6267/mu g/l, p=0.020, n=10). Leukocyte count was significantly lower in the CsA responders (1940.0 +/- 972.3/mu l) than in the non-responders (3253 +/- 2198/mu l, p=0.034). Conclusion 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.