Macrophage activation syndrome treatment, symptoms, life. Jul 20, 2018 pathways reported for macrophage activation syndrome mas development, a severe lifethreating complication of aosd 2,28. Macrophage activation syndrome angelo ravelli, md macrophage activation syndrome mas is 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 often considered a type of secondary hemophagocytic lymphohistiocytosis hlh and results from overactivation of t lymphocytes and macrophages leading to a cytokine storm. The administration of corticosteroid and cyclosporine a improved her respiratory depression along with all other symptoms. Apart from these inflammatory disorders, different autoinflammatory dysfunctions like kawasaki disease, systemic lupus erythematosus, and periodic fever syndromes can trigger the onset of adult and childhood macrophage activation syndrome. Jan 17, 2017 macrophage activation syndrome mas is a severe complication of rheumatic disease in childhood, particularly in systemic juvenile idiopathic arthritis sjia. Macrophage activation syndrome mas pediatric focus. Macrophage activation syndrome mas is the term used to describe a potentially lifethreatening complication of systemic inflammatory disorders, which occurs most commonly in systemic juvenile idiopathic arthritis jia and in its adult equivalent, adultonset stills disease 14, although its occurrence in patients.
Rowell syndrome complicated with macrophage activation. Macrophage activation syndrome mas, a form of secondary hemophagocytic lymphohistiocytosis, is a frequently fatal complication of a variety of pediatric inflammatory disorders. What does macrophage activation syndrome mean in english. Although the reported mortality rates for mas reach 20%, the clinical presentation of macrophage activation syndrome mas is generally acute and occasionally dramatic. Treatment of macrophage activation syndrome mas with. Evidencebased diagnosis and treatment of macrophage. Macrophage definition of macrophage by medical dictionary. Macrophage activation syndrome mas is an increasingly recognized complication of sle, although its true prevalence in childhood is still unknown. Macrophage activation syndrome is thought to be caused by excessive activation and proliferation of t lymphocytes and macrophages. Respiratory failure and macrophage activation syndrome as an. Definition of macrophage activation syndrome in the dictionary. We describe findings in mouse models of secondary hlh, comparing them with models of p.
The main manifestations of mas include fever, hepatosplenomegaly, lymphadenopathy, severe cytopenias, serious liver disease, and disseminated. This is usually on both sides and is painless but often get very. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. Information and translations of macrophage activation syndrome in the most comprehensive dictionary definitions resource on the web. Normally, when the immune system is activated, it fights the infection in a coordinated and controlled manner. It is characterize by an uncontrolled activation and proliferation of t lymphocytes and macrophages. The clinical features include a persistent highgrade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsislike condition. Hemophagocytic syndrome, hemophagocytic lymphohistiocytosis hlh, histiocytic erythrophagocytosis. Macrophage activation syndrome an overview sciencedirect. In individuals with macrophage activation syndrome mas, the immune system is uncontrolled and works in overdrive, leading to too much inflammation in the entire body. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for macrophage activation syndrome skip to main content u. We report a 36yearold chinese woman diagnosed with systemic lupus erythematosus who initially presented with macrophage activation syndrome mas and manifested respiratory failure.
Macrophage activation syndrome mas is an unusual, hyperinflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multiorgan. Macrophage activation syndrome mas is a lifethreatening condition, and it is a subset of hemophagocytic lymphohistiocytosis hlh. Falling esr and hyperferritinemia in the appropriate clinical setting can be. Nov 21, 2018 macrophage activation syndrome is characterized by a highly stimulated but ineffective immune response. Macrophage activation syndrome is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms and is. Mas is a severe, potentially fatal disease characterized by severe systemic inflammation. Macrophage activation syndrome is one of the rare causes of puo. Standardized diagnostic and treatment guidelines for mas in sjia are currently lacking. By analyzing complex interactions of the immune system in. So far there are 95 cases reported in the literature. Aug 22, 20 over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Pdf intravenous immunoglobulin treatment for macrophage. Macrophage activation definition of macrophage activation.
It occurs most commonly with systemiconset juvenile idiopathic arthritis sojia. Macrophage activation syndrome mas is a potentially fatal condition. There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome. It is characterised by pancytopenia, liver dysfunction, coagulopathy and neurological symptoms. Macrophage activation syndrome mas is a lifethreatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of t lymphocytes and. Hlh is not a single disease but is a hyperinflammatory syndrome that can occur in. Macrophage activation syndrome mas is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to systemic juvenile idiopathic arthritis sjia. Nov 21, 2018 macrophage activation syndrome mas is a lifethreatening complication of rheumatic disease that, for unknown reasons, occurs much more frequently in individuals with systemic juvenile idiopathic arthritis sjia and in those with adultonset still disease. In pediatric rheumatology, the term macrophage activation syndrome mas refers to a condition caused by excessive activation and expansion of t lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity.
Macrophage activation syndrome mas is a potentially lifethreatening complication of sjia as well as sometimes complicating other rheumatologic conditions, such as juvenile systemic lupus erythematosus thought to result from immune activation of pathogenic t cells and hemophagocytic macrophages. Macrophage activation syndrome mas is the term used to describe a potentially lifethreatening complication of rheumatic diseases, which is seen most frequently in systemic juvenile idiopathic arthritis sjia and in its adult equivalent, adultonset stills disease,, although it is encountered with increasing frequency in systemic lupus erythematosus of either childhood. Macrophage activation syndrome treated with anakinra the. Cytokines mediate most of the neurological symptoms. Macrophage activation syndrome clinical presentation. In adults, macrophage activation syndrome develops due to the complication of stills disease. Pdf macrophage activation syndrome triggered by epstein. Rheumaknowledgy macrophage activation syndrome mas. Sobi investigator initiated clinical trial of anakinrato treat mas case of. In addition, mas has been described in association with systemic lupus erythematosus sle, kawasaki disease, and adult. There are similarities in genetic background, pathogenesis, and clinical and laboratory features with primary hlh phlh.
However, its pathogenesis is still poorly understood and has many similarities with that of the other forms of hemophagocytic lymphohistiocytosis hlh. Mas is a lifethreatening condition associated with high mortality rates. The pathological mechanisms of mas are not fully understood. Within 48 hours of anakinra treatment, blood count increased and fever abated. Rosaidorfman disease symptoms histiocytosis association. Macrophage activation syndrome mas is a lifethreatening complication of rheumatic disease that, for unknown reasons, occurs much more frequently in individuals with systemic juvenile idiopathic arthritis sjia and in those with adultonset still disease. The differential diagnosis of hlh includes secondary hlh and macrophage activation syndrome or other primary immunodeficiencies that present with hemophagocytic lymphohistiocytosis, such as xlinked lymphoproliferative disease. Macrophage activation syndrome as onset of systemic lupus. Recent findings in hemophagocytic lymphohistiocytosis, a disease that. Macrophage activation syndrome is a rare and potentially fatal disease, to be considered in patients with a sirs like clinical presentation.
Other conditions that may be confused with this condition include autoimmune lymphoproliferative syndrome. Sep 24, 2015 macrophage activation syndrome mas is a potentially lifethreatening complication of rheumatic diseases such as systemic juvenile idiopathic arthritis sjia and systemic lupus erythematosus. Macrophage activation syndrome mas is a potentially fatal complication of rheumatic diseases. Another term for this form of hlh is reactive hemophagocytic syndrome. Rosaidorfman may affect lymph nodes only, it can affect lymph nodes plus other body systems, or it can occur in body systems without lymph node involvement.
Macrophage activation syndrome mas is a potentially lifethreatening complication of rheumatic diseases such as systemic juvenile idiopathic arthritis sjia and systemic lupus erythematosus. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. The array of processes that upregulate macrophage activity. Dec 01, 2010 in pediatric rheumatology, the term macrophage activation syndrome mas refers to a condition caused by excessive activation and expansion of t lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity. Macrophage activation syndrome genetic and rare diseases. Macrophage activation syndrome kill or be killed randy q. Macrophage activation syndrome page 2 of 7 the rheumatologist. They are components of the reticuloendothelial system. May 23, 2016 macrophage activation syndrome mas is a disorder whereby the immune system generates very high levels of substances cytokines that promote inflammation to the extent dysfunction occurs in multiple organ systems which if unchecked, is frequently fatal to the affected individual. Due to the low incidence of this disease, the epidemiology of rs is not clear. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients.
Macrophage activation syndrome mas is a severe, potentially fatal condition associated with excessive activation of macrophages and t cells leading to an overwhelming inflammatory reaction. In clinically similar primary hlh, the uncontrolled proliferation of t cells and macrophages has been linked to decreased natural killer nk cell and cytotoxic t cell function, often due to mutations in the gene encoding perforin. Pdf macrophage activation syndrome rakshita aggarwal. Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus. Macrophage activation syndrome in a child with systemic onset. Macrophage activation syndrome is a severe, potentially lifethreatening, complication of several chronic rheumatic diseases of childhood. Anakinra 100 mg subcutaneously daily was initiated, along with pulse methylprednisolone 1 gday for 3 days, then prednisone 25 mg twice daily. Macrophage activation syndrome mas is a lifethreatening complication of sjia marked by sudden onset of nonremitting high fever, profound depression in all three blood cell lines i. There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome mas, adult onset stills disease aosd, catastrophic antiphospholipid syndrome caps and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. A diagnosis of macrophage activation syndrome mas was made. The condition is considered part of secondary hemophagocytic lymphohistiocytoses hlh. Macrophage activation syndrome in systemic juvenile. Mas is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis shlh.
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