Macrophage activation syndrome: A severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients

2016 ◽  
Vol 45 (6) ◽  
pp. 706-710 ◽  
Author(s):  
Natali W.S. Gormezano ◽  
Carini I. Otsuzi ◽  
Diego L. Barros ◽  
Mariana A. da Silva ◽  
Rosa M.R. Pereira ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Acute Pancreatitis ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus ◽  
Activation Syndrome

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

2018 ◽  
Vol 5 (4) ◽  
pp. 1
Author(s):  
Cyrus Ashraf Askin ◽  
Jerome Craig Edelson ◽  
Guy Smith Dooley ◽  
Amy Nicole Stratton
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Acute Pancreatitis ◽  
Inflammatory Response ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Necrotizing Pancreatitis ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Activation Syndrome

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.

scholarly journals Case Report: Macrophage Activation Syndrome and Widespread Neuroimaging Abnormality in Childhood-Onset Systemic Lupus Erythematosus

2021 ◽  
Vol 9 ◽  
Author(s):  
Nana Shi ◽  
Xiangying Wang ◽  
Lixia Zou ◽  
Xinghui Yang ◽  
Qian Ma ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Resonance Imaging ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) and widespread brain lesions are rare and severe complications of childhood-onset systemic lupus erythematosus (SLE). We report an 11-year-old girl who presented with recurrent rashes for half a year and fever for 2 weeks. Clinical and laboratory features at admission pointed to the diagnosis of SLE and SLE-associated MAS. Cerebral magnetic resonance imaging taken on day 4 after admission showed abnormal signals. Glucocorticoid therapy was started on day 5. Two days later, the patient appeared weak and ill, then the next day she exhibited dizziness, drowsiness, apathia, and dysarthria. High-dose methylprednisolone, cyclophosphamide, and intravenous immunoglobulin were used to treat the patient, and intrathecal dexamethasone was given. The patient was discharged on day 30 after admission and showed complete clinical resolution and improved magnetic resonance imaging resolution.

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