Macrophage activation syndrome.
Summary of "Macrophage activation syndrome."
Macrophage activation syndrome (MAS) is an infrequent but potentially life-threatening complication of various autoimmune diseases. It is characterized by excessive activation of macrophages induced by a dysfunction of the immune system, although the underlying abnormalities remain to be delineated. Sepsis-like symptoms accompanied by cytopenia, hepatosplenomegaly, coagulopathy and multiple organ dysfunctions are typical disease manifestations. Due to the lack of validated classification criteria and distinct laboratory markers, the diagnosis of MAS is often difficult to establish. However, early diagnosis and therapy is of utmost importance to ensure best possible outcome. Treatment regimens include high doses of glucocorticoids, classical immunosuppressive agents as well as novel biologics.
Affiliation
Klinik und Poliklinik für Rheumatologie/Klinische Immunologie, Asklepios Klinik Bad Abbach, Kaiser-Karl V.-Allee 3, 93077, Bad Abbach, Deutschland, pe.lehmann@asklepios.com.
Journal Details
This article was published in the following journal.
Name: Zeitschrift fur Rheumatologie
ISSN: 1435-1250
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22836385
- DOI: http://dx.doi.org/10.1007/s00393-012-0987-7
Medical and Biotech [MESH] Definitions
Macrophage Activation
The process of altering the morphology and functional activity of macrophages so that they become avidly phagocytic. It is initiated by lymphokines, such as the macrophage activation factor (MAF) and the macrophage migration-inhibitory factor (MMIF), immune complexes, C3b, and various peptides, polysaccharides, and immunologic adjuvants.
Macrophage Activation Syndrome
A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS.
Macrophage Colony-stimulating Factor
A mononuclear phagocyte colony-stimulating factor (M-CSF) synthesized by mesenchymal cells. The compound stimulates the survival, proliferation, and differentiation of hematopoietic cells of the monocyte-macrophage series. M-CSF is a disulfide-bonded glycoprotein dimer with a MW of 70 kDa. It binds to a specific high affinity receptor (RECEPTOR, MACROPHAGE COLONY-STIMULATING FACTOR).
Particulate Matter
Particles of any solid substance, generally under 30 microns in size, often noted as PM30. There is special concern with PM1 which can get down to PULMONARY ALVEOLI and induce MACROPHAGE ACTIVATION and PHAGOCYTOSIS leading to FOREIGN BODY REACTION and LUNG DISEASES.
Macrophage-activating Factors
Factors secreted by stimulated lymphocytes that prime macrophages to become nonspecifically cytotoxic to tumors. They also modulate the expression of macrophage cell surface Ia antigens. One MAF is INTERFERON-GAMMA. Other factors antigenically distinct from IFN-gamma have also been identified.
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