Clinical analysis of Kawasaki disease associated with macrophage activation syndrome and a probe into its diagnostic criteria / 中华实用儿科临床杂志

ABSTRACT

Objective To analyze the clinical and laboratory characteristics,treatment,and outcomes of Ka-wasaki disease (KD)patients associated with macrophage activation syndrome (MAS)(KD - MAS)and to compare three diagnostic standards. Methods Twelve cases of KD - MAS were reviewed retrospectively,who had been treated and therapied at the Children′s Hospital of Chongqing Medical University from September 2007 to September 2017. The clinical data were analyzed. And,the efficacy of different MAS diagnostic criteria for KD - MAS was evaluated. Results The subjects included 8 males and 4 females,with a median age of 25 months. The capital trigger of MAS was infection(8 cases,66. 7％). Unabating high fever had been the initial manifestation for 12 patients(100％),other com-mon clinical features including hepatomegaly(11 cases,91. 6％),splenomegaly(8 cases,66. 7％)and lymphadenectasis (7 cases,58. 3％). Besides,8 patients (66. 7％)had different degrees of central nervous system symptoms. Laboratory examination showed a decrease in hemoglobin (11 cases,91. 6％),in thrombocytopenia (8 cases,66. 7％),and white blood cells (4 cases,33. 3％);while there was an increase were found in serum transaminase (11 cases,91. 6％), triglyceride(72. 7％,8 / 11 cases)and serum ferritin (100％,9 / 9 cases). Eleven patients (91. 6％)had decreased erythrocyte sedimentation rates (ESR). Bone marrow cytology was performed in 10 cases,and 8 cases of them showed hemophagocytic phenomenon. All the patients were diagnosed by SoJIA - MAS(2005)criteria. All patients were treated with high - dose intravenous immunoglobulin (IVIG)treatment,among whom 3 cases were combined with methylpred-nisolone treatment,and 2 cases received with more than 2 kinds of immunosuppressive drugs (Dexamethasone and Ciclosporin or Etoposide). Among the 12 patients,2 patients lost to follow - up,4 cases(33. 3％)died due to hepatic encephalopathy,including 2 cases who withdrawn treatment the remaining 6 cases (50. 0％)improved. Conclusions Prolonged high fever is the first manifestation of MAS in KD. Hemogram and ESR will decrease,elevated serum transaminase and ferritin may increase,which indicates MAS occurrence. If a high dose of IVIG therapy does not work,the combination of glucocorticoid and immunosuppressive for therapy may improve the remission rate. Severe cen-tral nervous system involvement may indicate a terrible prognosis. SoJIA - MAS (2005)can diagnose earliler by using preliminary diagnostic guidelines for macrophage activation system complicating systemic juvenile idiopathic arthritis.