BackgroundMacrophage activation syndrome (MAS) is the secondary hemophagocytic lymphohistiocytosis associated with rheumatic diseases. Recently, the different cytokine profiles between systemic juvenile idiopathic arthritis (sJIA)-associated MAS (sJIA-MAS) and juvenile systemic lupus erythematosus (JSLE)-associated MAS (JSLE-MAS) were reported. However, there is little information about juvenile dermatomyositis (JDM)-associated MAS (JDM-MAS).Case presentationA 4-year-old girl with JDM was hospitalized because of fever, erythema, hepatosplenomegaly, cytopenia, liver dysfunction and coagulopathy. Bone marrow aspiration revealed appreciable numbers of activated and hemophagocytosing macrophages. She was diagnosed as having JDM–MAS complicated with interstitial pneumonia (IP) based on the findings of the elevation of serum Krebs von den Lungen-6 (KL-6) levels and chest computed tomography findings. We analyzed circulating levels of interleukin (IL)-2,4,6,10,18, tumor necrosis factor-α and interferon-γ in the patient. Hypercytokinemia occurred at the diagnosis of MAS and IP, showing with the prominent elevations of IL-6 and IL-18 levels. The cytokine profiles were distinct from those reported in patients with sJIA-MAS or JSLE-MAS. High-dose corticosteroid and cyclosporine therapy led to a drastic improvement of MAS with decreased IL-6 levels. Subsequent cyclophosphamide therapy successfully controlled IP, paralleled with the declining pattern of IL-18 and KL-6 levels.ConclusionThis is the first report to describe a successful treatment and the cytokine profile of JDM-MAS and IP. Serum IL-6 and IL-18 levels may be useful for predicting the disease activity of JDM-MAS and IP, respectively.
We report the second case of the association of Kawasaki disease (KD) and autoimmune neutropenia (AIN). A 21-month-old female diagnosed as having AIN of infancy developed a complete KD when severe neutropenia continued. The patient suffered from no coronary artery lesions, and well responded to a single high-dose gamma-globulin therapy. The cytokine profile of the neutropenic infant was representative of the typical KD. Neutrophil counts notably increased during the convalescent phase of KD, and were then normalized forthwith. The prompt resolutions of KD and AIN paralleled the increase of circulating transforming growth factor (TGF)-β1 levels. The clinical course of the patient was contrasted to that of the first reported case of a patient who developed severe and refractory KD after the high dose granulocyte-colony stimulating factor (G-CSF) therapy.
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