Macrophage Activation Syndrome Treated with Anakinra

Durand, Madéleine; Troyanov, Yves; Laflamme, Pierre; Grégoire, Geneviève

doi:10.3899/jrheum.091046

Cited by 82 publications

(50 citation statements)

References 8 publications

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“…Intravenous immunoglobulin was administered to a sizeable proportion of the patients, probably owing to both its immunoregulatory properties and its potential to aid in preventing septic complications. Biologic medications were given to 15.2% of the patients, with anakinra being the most frequently selected, likely due to recent reports of its dramatic effectiveness in MAS (4), including patients whose disease was refractory to high-dose corticosteroids and cyclosporine (36)(37)(38)(39). Etoposide, which is a central medication in HLH therapeutic protocols (40), was used in 11% of cases.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

Minoia

Davì

Horne

et al. 2014

Arthritis & Rheumatology

356

298

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Objective To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). Methods In this multinational, multicenter study, pediatric rheumatologists and hemato‐oncologists entered patient data collected retrospectively into a web‐based database. Results A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and d‐dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre‐MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one‐third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%. Conclusion This study provides information on the clinical spectrum and current management of systemic JIA–associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.

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Section: Discussionmentioning

confidence: 99%

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

Minoia

Davì

Horne

et al. 2014

Arthritis & Rheumatology

356

298

View full text Add to dashboard Cite

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“…1 Anakinra has been used with positive response in patients with HLH secondary to Systemic Juvenile Idiopathic Arthritis, Adult Still's disease, Systemic Lupus Erythematosus, and even Cytomegalovirus infection. 1,3,8,9,11 It is clear that not all adult cases of HLH require initiation of the complete HLH-2004 protocol treatment, which includes chemotherapeutics, as these medications carry harmful side effects. 2 Use of a biological response modifier along with dexamethasone may achieve treatment of secondary HLH in an adult patient as exemplified by this case.…”

Section: Discussionmentioning

confidence: 99%

“…Overall, our patient's presentation was in line with documented cases of adult HLH in that our patient had a fever (93% of cases), splenomegaly (50% of cases), elevated ferritin (98% of cases), and hemophagocytosis seen on bone marrow biopsy (97% of cases). 4,[8][9][10][11] Given that diagnosis remains a barrier to HLH treatment, future work should focus on developing adult specific diagnostic criteria. In 2014, a research group based in Paris constructed a diagnostic score called the "HScore" which may be used to estimate an individual's risk for reactive hemophagocytic syndrome according to weighted criteria.…”

Section: Discussionmentioning

confidence: 99%

A Case of Secondary Hemophagocytic Lymphohistiocytosis in an Adult Patient Treated with Concurrent Dexamethasone and Interleukin-1 Receptor Blockade

Sunder¹

2018

TMF

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“…Anakinra is an IL-1 antagonist, which has been successfully used in cases of MAS. [5] Adult cases of SLE-associated AP complicated by MAS are very rare. We present a unique case of and adult with SLE-associated AP who ultimately developed MAS requiring anakinra.…”

Section: Introductionmentioning

confidence: 99%

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Askin

Edelson

Dooley

et al. 2018

CRIM

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Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

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Macrophage Activation Syndrome Treated with Anakinra

Cited by 82 publications

References 8 publications

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

A Case of Secondary Hemophagocytic Lymphohistiocytosis in an Adult Patient Treated with Concurrent Dexamethasone and Interleukin-1 Receptor Blockade

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

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