Severe macrophage activation syndrome and central nervous system involvement in juvenile dermatomyositis

Lilleby, Vibke; Haydon, Jo; Sanner, Helga; Krossness, BK; Ringstad, Geir; Flatø, Berit

doi:10.3109/03009742.2013.863968

Cited by 21 publications

(8 citation statements)

References 8 publications

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“…There has been no report about the cytokine profile in JDM-MAS with IP. To our knowledge, only 5 patients with JDM-MAS were reported [ 4 , 7 – 10 ]. There were 4 boys and 1 girl, the median age of whom was 12 years ranging from 7 to 14.…”

Section: Discussionmentioning

confidence: 99%

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Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

et al. 2015

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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.

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Section: Discussionmentioning

confidence: 99%

“…Juvenile systemic lupus erythematosus (JSLE) is also one of the underlying rheumatic diseases in which MAS occurs more frequently seen than in the others [ 3 ]. On the other hand, MAS is a quite rare complication in JDM patients [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

et al. 2015

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“…Gastrointestinal and pulmonary involvement are also common [2,3]. Other systemic manifestations including pseudo-Purtscher's retinopathy [4,5] and JDM-associated MAS [5][6][7] have been reported as well, but are quite rare.…”

Section: Discussionmentioning

confidence: 99%

The unusual suspects: a curious case of acute kidney injury—Answers

2019

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“…MAS has been associated with other rheumatic diseases, including systemic lupus erythematosus (SLE) , Kawasaki disease , spondyloarthritis , and juvenile dermatomyositis (DM) . As these are rarer disorders than systemic JIA–associated MAS, reports are scarce and systematic studies are limited.…”

Section: Pathogenesis Of Cytokine Storm Syndromesmentioning

confidence: 99%