Abstract
Macrophage activation syndrome (MAS) is a life-threatening complication of systemic inflammatory disorders that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important and recently preliminary diagnostic guidelines for the syndrome have been proposed. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to propose to rename it according to the contemporary classification of histiocytic disorders. Cyclosporine A has been found to be effective in patients with corticosteroid resistant MAS.
| Original language | English |
|---|---|
| Pages (from-to) | 225-230 |
| Number of pages | 6 |
| Journal | Current Rheumatology Reviews |
| Volume | 3 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Aug 2007 |
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Keywords
- Hemophagocytic lymphohistiocytosis
- Hemophagocytic syndrome
- Macrophage activation syndrome
- Systemic juvenile idiopathic arthritis
ASJC Scopus subject areas
- Rheumatology
Cite this
Macrophage activation syndrome in childhood rheumatic diseases. / Ravelli, Angelo; Pringe, Alejandra; Malattia, Clara; Sala, Ilaria; Martini, Alberto.
In: Current Rheumatology Reviews, Vol. 3, No. 3, 08.2007, p. 225-230.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Macrophage activation syndrome in childhood rheumatic diseases
AU - Ravelli, Angelo
AU - Pringe, Alejandra
AU - Malattia, Clara
AU - Sala, Ilaria
AU - Martini, Alberto
PY - 2007/8
Y1 - 2007/8
N2 - Macrophage activation syndrome (MAS) is a life-threatening complication of systemic inflammatory disorders that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important and recently preliminary diagnostic guidelines for the syndrome have been proposed. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to propose to rename it according to the contemporary classification of histiocytic disorders. Cyclosporine A has been found to be effective in patients with corticosteroid resistant MAS.
AB - Macrophage activation syndrome (MAS) is a life-threatening complication of systemic inflammatory disorders that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important and recently preliminary diagnostic guidelines for the syndrome have been proposed. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to propose to rename it according to the contemporary classification of histiocytic disorders. Cyclosporine A has been found to be effective in patients with corticosteroid resistant MAS.
KW - Hemophagocytic lymphohistiocytosis
KW - Hemophagocytic syndrome
KW - Macrophage activation syndrome
KW - Systemic juvenile idiopathic arthritis
UR - http://www.scopus.com/inward/record.url?scp=34548363286&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=34548363286&partnerID=8YFLogxK
U2 - 10.2174/157339707781387617
DO - 10.2174/157339707781387617
M3 - Article
VL - 3
SP - 225
EP - 230
JO - Current Rheumatology Reviews
JF - Current Rheumatology Reviews
SN - 1573-3971
IS - 3
ER -