Macrophage Activation Syndrome in Adults: A Retrospective Case Series

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

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Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

Case Reports in Pediatrics ◽

10.1155/2017/5693501 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Kenan Barut ◽

Sezgin Sahin ◽

Amra Adrovic ◽

Velat Sen ◽

Ozgur Kasapcopur

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Inflammatory Diseases ◽

Delayed Diagnosis ◽

Systemic Juvenile Idiopathic Arthritis ◽

Pulmonary Hemosiderosis ◽

Pulmonary Manifestations ◽

Life Threatening ◽

Activation Syndrome

Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

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Higher-dose canakinumab therapy for refractory macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: two case reports

Pediatrician (St Petersburg) ◽

10.17816/ped5414-19 ◽

2014 ◽

Vol 5 (4) ◽

pp. 14-19 ◽

Cited By ~ 1

Author(s):

Mikhail Mikhaylovich Kostik ◽

Tatyana Serafimovna Likhacheva ◽

Irina Aleksandrovna Chikova ◽

Natal’ya Valer’yevna Buchinskaya ◽

Natal’ya Nikolaevna Abramova ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Case Reports ◽

Interleukin 1 ◽

Systemic Juvenile Idiopathic Arthritis ◽

Fatal Complication ◽

Cytokine Inhibition ◽

Life Threatening ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a life-threatening, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) appears in non-remitted fever, cytopenia, coagulopathy, liver and CNS dysfunctions. Triggers of MAS could be disease activity, infections and medications. Known IL-1 is the key cytokine in pathogenesis of MAS and SJIA, and disease flare associated with increased amounts of different cytokines, especially IL-1β. Many cases of MAS are medically-refractory to traditional doses of cytokine inhibition and may require increased dosing of biologic cytokine blockade. Interleukin-1 (IL-1) is typically a key cytokine in the pathogenesis of sJIA and associated MAS. When MAS occurs in the setting of sJIA treated with IL-1 inhibitors, then increased dosing of IL-1 blockers may be beneficial. This has been shown for anakinra, an IL-1 receptor antagonist, but this drug is currently not available worldwide. Another IL-1 blocker, canakinumbab (CKB), is a monoclonal antibody that blocks IL-1β, but does not also block IL-1α like anakinra. Herein, we describe 2 sJIA patients who developed MAS on standard doses of CKB (4 mg/kg). Both patients received an increased dose of CKB: 150 mg (7.5 and 12.5 mg/kg, respectively) with rapid and complete resolution of MAS. Later the CKB doses was tapered to normal regimen. No side effects or adverse events were noticed during usage of increased CKB doses. Increased dosing of CKB should be considered for CKB-treated sJIA patients who develop MAS on standard dosing.

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Hyperferritinemia Wins Again: Defining Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus

The Journal of Rheumatology ◽

10.3899/jrheum.210024 ◽

2021 ◽

pp. jrheum.210024

Author(s):

Emily A. Smitherman ◽

Randy Q. Cron

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Systemic Juvenile Idiopathic Arthritis ◽

Systemic Lupus ◽

Pediatric Systemic Lupus Erythematosus ◽

Threatening Condition ◽

Life Threatening ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially life-threatening condition of hyperinflammation that can be secondary to an underlying chronic rheumatic condition, commonly systemic juvenile idiopathic arthritis (sJIA) but also childhood-onset systemic lupus erythematosus (cSLE). MAS is characterized by excessive activation of T lymphocytes and macrophages that lead to overproduction of cytokines and results in cytopenia, liver dysfunction, and coagulopathy1.

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Macrophage activation syndrome

Oxford Textbook of Rheumatology ◽

10.1093/med/9780199642489.003.0168_update_001 ◽

2013 ◽

pp. 1442-1446

Author(s):

Alexei A. Grom ◽

Athimalaipet V. Ramanan

Keyword(s):

T Lymphocytes ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Systemic Juvenile Idiopathic Arthritis ◽

Optimal Level ◽

Underlying Disease ◽

Threatening Condition ◽

Life Threatening ◽

Well Differentiated ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a life-threatening condition caused by excessive activation and proliferation of T lymphocytes and haemophagocytic macrophages. Although MAS has been reported in association with almost any rheumatic disease, it is by far most common in systemic juvenile idiopathic arthritis. Flares of the underlying disease or infection are most common triggers of MAS. The pathognomonic feature of MAS is typically found in bone marrow: numerous, well-differentiated macrophagic histiocytes phagocytosing normal haematopoietic elements. The expansion of these histiocytes leads to a massive systemic inflammatory reaction associated with three cardinal clinical features: severe cytopenias, liver dysfunction, and coagulopathy consistent with disseminated intravascular coagulation. Clinically, MAS is strikingly similar to the autosomal recessive disorders collectively known as familial haemophagocytic lymphohistiocytosis (FHLH). FHLH has been associated with various genetic defects affecting the cytolytic pathway. Cytolytic function is profoundly depressed in MAS patients as well, and this abnormality is caused by both genetic and acquired factors. Studies in animals suggest that uncontrolled expansion of activated CD8+ T lymphocytes secreting cytokines that activate macrophages is central to the pathophysiology of haemophagocytic syndromes. Consistent with this view, the combination of steroids and ciclosporin, an immunosuppressant that preferentially inhibits T lymphocytes, is an effective treatment for the majority of MAS patients. Patients in whom MAS remains active despite this treatment present a serious challenge and require more aggressive immunosuppression. However, in MAS triggered by infection, the optimal level of immunosuppression is difficult to determine. As a result, reported mortality rates reach 20%.

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Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report

Journal of Nepal Medical Association ◽

10.31729/jnma.7019 ◽

2021 ◽

Vol 59 (243) ◽

pp. 1166-1169

Author(s):

Rishikesh Kafle ◽

Anwesh Bhatta ◽

Sumit Gami ◽

Abhin Sapkota ◽

Dipesh Sharma ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Delayed Diagnosis ◽

Systemic Juvenile Idiopathic Arthritis ◽

Intermittent Fever ◽

Impaired Liver ◽

Threatening Condition ◽

Life Threatening ◽

Activation Syndrome

Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.

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