Macrophage activation syndrome mas is a lifethreatening condition, and it is a subset of hemophagocytic lymphohistiocytosis hlh. Hemophagocytic syndrome, hemophagocytic lymphohistiocytosis hlh, histiocytic erythrophagocytosis. Macrophage activation syndrome as an initial presentation. In pediatric rheumatology, the term macrophage activation syndrome mas refers to a condition caused by excessive activation and expansion. It remains a rare pathology, with a guarded prognosis, characterized by clinical and biological signs that are not very specific and whose association helps invoke the diagnosis. Macrophage activation syndrome is characterized by pancytopenia, liver insufficiency. Haemophagocytic lymphohistiocytosis hlh is an underrecognized hyperinflammatory condition with a high mortality, characterized by inappropriate survival of histiocytes and cytotoxic t cells ctls, leading to a cytokine storm, haemophagocytosis and multiorgan damage. In addition, mas has been described in association with systemic lupus erythematosus sle, kawasaki disease, and adult. The genetics, clinical features, and diagnosis of hlh are presented separately. A subanalysis of a randomized clinical trial indicated sepsis survival benefit from interleukin il1 blockade in patients with features of the macrophage activationlike syndrome mals. Recent findings in hemophagocytic lymphohistiocytosis, a disease that.
Macrophage activation syndrome mas is a rare and potentially fatal disorder, thought to result from uncontrolled activation and proliferation of t cells and excessive activation of macrophages. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients. After extensive workup and lack of improvement with broadspectrum antimicrobial therapy, intravenous immunoglobulin and steroids, the patient was diagnosed with macrophage activation syndrome after bone marrow biopsy and levels of soluble cd25 soluble ncbi. Macrophage activation syndrome mas is a rare, lifethreatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Current focus has been on the development of novel therapeutics, including antivirals and vaccines. In the case of severe case, the intravenous route is usually chosen for administering the drug. The treatment and prognosis of patients with hlh and the macrophage activation syndrome mas, a form of hlh in patients with juvenile idiopathic arthritis and other rheumatologic clinical manifestations and diagnosis of adult stills disease view in chinese. The mainstay of mas treatment is glucocorticoid therapy. Depending upon the different medical literature the treatment of macrophage activation syndrome are as follows. Predictive factors and prognosis of macrophage activation syndrome associated with adultonset stills disease. Evidencebased diagnosis and treatment of macrophage activation. Macrophage activation syndrome genetic and rare diseases.
Treatment of macrophage activation syndrome mas with. Synonymous with secondary hemophagocytic lymphohistiocytosis, macrophage activation syndrome mas is a term used by rheumatologists to describe a potentially lifethreatening complication of systemic inflammatory disorders, most commonly systemic juvenile idiopathic arthritis sjia and systemic lupus erythematosus sle. The term mas designates a clinicopathologic entity that occurs in different hemophagocytic syndromes hss. 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. Macrophage activation syndrome is the dysregulation of macrophagelymphocyte interactions leading to uncontrolled proliferation and activation of macrophages and t cells. This can occur in the setting of a number of different immune. The treatment and prognosis of patients with hlh and the macrophage activation syndrome mas, a form of hlh in patients with juvenile idiopathic arthritis and other rheumatologic conditions, will be discussed here. Evidencebased diagnosis and treatment of macrophage. In macrophage activation syndrome, macrophages are excessively activated and cant be shut down. Prognostic factors of macrophage activation syndrome, at the. To summarise the clinical data of adultonset stills disease aosd patients and analyse their clinical manifestations, predictors for the formation and prognosis of macrophage activation syndrome mas.
Prognosis depends on the severity of neurologic manifestations. Review macrophage activation syndrome as part of systemic juvenile idiopathic arthritis. Nov 01, 2019 macrophage activation syndrome mas is a rare and severe complication of kawasaki disease kd. A febrile patient with known or suspected sjia must be considered for macrophage activation if. Macrophage activation syndrome treatment, symptoms, life.
There are few reports of longterm outcomes of mas complicating csle. Anakinra 100 mg subcutaneously daily was initiated, along with pulse methylprednisolone 1 gday for 3 days, then prednisone 25 mg twice daily. Soluble hemoglobinhaptoglobin scavenger receptor cd163 as a lineagespecific marker in. 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 the clinical utility of splenic fluorodeoxyglucose. Macrophage activation syndrome mas is a phenomenon characterized by cytopenia, organ dysfunction, and coagulopathy associated with an inappropriate activation of macrophages 1.
It is sometimes difficult to establish the diagnosis of hemophagocytic lymphohistiocytosis hlh, and the combination of the physical symptoms and certain laboratory tests is required. Macrophage activation syndrome mas is a lifethreatening inflammatory complication of childhoodonset systemic lupus erythematosus csle. Macrophage activation syndrome an overview sciencedirect. If you have questions about which treatment is right for you, talk to your healthcare professional. Macrophage activation syndrome mas is a term used by rheumatologists to describe a potentially lifethreatening complication of systemic inflammatory disorders, most commonly systemic juvenile idiopathic arthritis sjia and its adult equivalent adult onset still disease and systemic lupus erythematosus sle 1. First line treatment for macrophage activation syndrome is considered as highdose corticosteroid therapy. 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 clinical features include a persistent highgrade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsislike condition. Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with munc4 polymorphisms. Macrophage activation syndrome, a rare complication of. Macrophage activation syndrome mas is a lifethreatening complication of rheumatic. There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome. 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. Apr 19, 2019 macrophage activation syndrome is a condition in which the bodys immune system activates excessively, leading to tissue and organ damage. Predictive factors and prognosis of macrophage activation. 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. A diagnosis of macrophage activation syndrome mas was made.
Patients with infections and systemic inflammatory response syndrome were assigned to. Frontiers the immunology of macrophage activation syndrome. It remains a rare pathology, with a guarded prognosis, characterized by clinical and biological signs that are not very specific and whose association helps invoke. Hemoglobin platelets x 109l neutrophils treatment available for macrophage activation syndrome. Predictive factors and prognosis of macrophage activation syndrome. Laboratory abnormalities of mas include pancytopenia, hyperferritinemia, hypertriglyceridemia and elevated serum. 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. It occurs most commonly with systemiconset juvenile idiopathic arthritis sojia. Boom v, anton j, lahdenne p, quartier p, ravelli a, wulffraat nm, et al. 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. 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 in adults is poorly studied. Diagnosis is a challenge, treatment has to be started early and be aggressive to reduce the.
Laboratory abnormalities of mas include pancytopenia, hyperferritinemia, hypertriglyceridemia and elevated serum transaminases. Macrophage activation syndrome treated with anakinra the. Macrophage activation syndrome in adults with rheumatic disease. Actemra can mask macrophage activation syndrome in.
Macrophage activation syndrome mas is a rare and severe complication of kawasaki disease kd. Soluble hemoglobinhaptoglobin scavenger receptor cd163 as a lineagespecific marker in the reactive hemophagocytic syndrome. Macrophage activation syndrome causes, symptoms, diagnosis. Macrophage activation syndrome mas is an intriguing and potentially lifethreatening condition 1, 2, clinically characterized by nonremitting fever, hepatosplenomegaly, lymphadenopathy, encephalopathy, coagulopathy and even multi organ failure in severe cases. Macrophage activation syndrome is the dysregulation of macrophage lymphocyte interactions leading to uncontrolled proliferation and activation of macrophages and t cells. Macrophages are a type of immune cell in the body that eats infectious organisms and damaged cells. We read with great interest the article by minoia et al which named development and initial validation of the macrophage activation syndrome massystemic juvenile idiopathic arthritis sjia ms score for diagnosis of mas in sjia. 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. Application of ms score in macrophage activation syndrome. Hlh is classified into familial fhlh and into secondary shlh.
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. The resources below provide information about treatment options for this condition. Type 2 has mutations in rab27a and haemophagocytic syndrome, with abnormal tcell and macrophage activation. Hemophagocytic syndrome, hemophagocytic lymphohistiocytosis hlh, histiocytic erythrophagocytosis icd9 code.
A set of diagnostic criteria was recommended by the histiocyte society for use in the hlh2004 research protocol, and this was revised in 2007. Macrophage activation syndrome mas is a phenomenon characterized by cytopenia, organ dysfunction, and coagulopathy associated with an inappropriate activation of macrophages. Drugs used to treat macrophage activation syndrome. Mas is a severe, potentially fatal disease characterized by severe systemic inflammation. Treating systemic juvenile idiopathic arthritis sjia with actemra tocilizumab can modify the clinical and laboratory features of macrophage activation syndrome mas a severe, potentially lifethreatening complication of rheumatic diseases and complicate its diagnosis, a study found as such, the researchers said, it is not appropriate to use standard mas classification criteria. Macrophage activation syndrome mas is address correspondence to dr wulffraat. Macrophage activation syndrome mas is a clinical syndrome caused by excessive activation and proliferation of well differentiated macrophages. Prognostic factors of macrophage activation syndrome, at.
Macrophage activation syndrome symptoms, treatments. Macrophage activation syndrome page 6 of 7 the rheumatologist. Aug 31, 2018 macrophage activation syndrome mas is a lifethreatening condition, and it is a subset of hemophagocytic lymphohistiocytosis hlh. The goal in treating mas is to reduce bodywide inflammation as quickly as possible and prevent organ damage. Evidencebased diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. In adults, macrophage activation syndrome develops due to the complication of stills disease. Macrophage activation syndrome as part of systemic. 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. Patients with adultonset still disease with macrophage activation. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. Macrophage activation syndrome is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms and is.
May 05, 2020 macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with munc4 polymorphisms. Symptoms include fever, a painful mass or masses in the abdomen, easy bruising or bleeding, swollen lymph nodes, a rash, headache, confusion, vision changes, seizures andor coma as well as difficulty breathing. 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. Our aim is to compare longterm outcomes between mas and nonmas patients with csle seen at a single tertiary centre. Clinical and laboratory features of mas include sustained fever. Longterm outcomes of macrophage activation syndrome in. The clinical feature, early diagnosis and treatment options, and prognosis need to be further determined in patients with kd complicated with mas. Adult macrophage activation syndrome mas, is a lifethreatening complication, developing during the clinical course of several inflammatory diseases such as adult onset stills disease aosd and systemic lupus erythematosus sle. This study aimed to investigate the frequency of mals and to develop a biomarker of diagnosis and prognosis. Common symptoms reported by people with macrophage activation syndrome. Apr 28, 2020 macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with munc4 polymorphisms. Defining criteria for macrophage activation syndrome, a. Macrophage activation syndrome is a serious complication of rheumatic disease, thought to be caused by excessive activation of macrophages and lymphocytes. 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.
The treatment of macrophage activation syndrome mas is traditionally based on the parenteral administration of high doses of corticosteroids. Macrophage activation syndrome mas aboutkidshealth. Over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Mas is a lifethreatening complication of rheumatic disorders, including sjia, adultonset stills disease aosd and lupus. Although macrophage disorders are usually a consequence of a decrease in function, an increase in macrophage activity can also have adverse consequences for individuals. Macrophage activation syndrome mas is a pathological systemic. Macrophage activation syndrome, by definition, occurs in people who have an underlying rheumatic disorder, or an autoimmune disorder of the joints and connective tissues. Haemophagocytic lymphohistiocytosis hlh is an aberrant hyperinflammatory, hyperferritinemic immune response syndrome driven by macrophages and cytotoxic t cells. Most of the physicians start with an intravenous methylprednisolone 30 mgkgdose. Rheumaknowledgy macrophage activation syndrome mas. Kawasaki disease complicated with macrophage activation.
The following list of medications are in some way related to, or used in the treatment of this. List of macrophage activation syndrome medications 1. Predictive factors and prognosis of macrophage activation syndrome associated with adultonset stills disease x. What is the prognosis of macrophage activation syndrome mas. Mas refers to reactive hemophagocytic lymphohistiocytosis hlh, which may be classified into primary, the genetic forms, and secondary, the. Jan 31, 2019 hlh is classified into familial fhlh and into secondary shlh. The inflammation can be in any organ system including the bone marrow, liver, and spleen. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically sim.
293 60 236 1183 534 1155 409 1088 1262 410 729 388 107 1014 136 522 790 69 828 1318 1386 1562 424 142 381 515 21 239 1145 78 245 1098 1052 838 1614 451 759 736 117 786 958 343 8 50 1245