scholarly journals Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Wesam Gouda ◽  
Faisal Alsaqabi ◽  
Abdelhfeez Moshrif ◽  
Awad S. Abbas ◽  
Tarek M. Abdel-Aziz ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Intravenous Immunoglobulin ◽  
Lupus Erythematosus ◽  
Sodium Phosphate ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Patients ◽  
Systemic Lupus ◽  
Dexamethasone Sodium Phosphate ◽  
Activation Syndrome

Abstract Background Macrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge. Case presentation Herein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response. Conclusion Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

scholarly journals Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1065-1071 ◽  
Author(s):  
E M Cohen ◽  
K D’Silva ◽  
D Kreps ◽  
M B Son ◽  
K H Costenbader
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hospital Admission ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activity Index ◽  
Conditional Logistic Regression ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Activation Syndrome

Background Macrophage activation syndrome (MAS) is an uncommon but serious complication of systemic lupus erythematosus (SLE). We aimed to identify factors associated with MAS among adult hospitalized SLE patients. Methods Within the Brigham and Women’s Hospital (BWH) Lupus Center Registry, we identified adult SLE patients > age 17 who had been hospitalized from 1970 to 2016, with either ferritin > 5000 ng/ml during admission or “macrophage activation syndrome” or “MAS” in discharge summary. We confirmed MAS by physician diagnosis in medical record review. We matched each hospitalized SLE patient with MAS to four SLE patients hospitalized without MAS (by SLE diagnosis date ±1 year). We employed conditional logistic regression models to identify clinical factors associated with MAS among hospitalized SLE patients. Results Among 2094 patients with confirmed SLE, we identified 23 who had a hospitalization with MAS and compared them to 92 hospitalized without MAS. Cases and controls had similar age at SLE diagnosis (29.0 vs. 30.5, p = 0.60), and hospital admission (43.0 vs. 38.3, p = 0.80), proportion female (78% vs. 84%, p = 0.55), and time between SLE diagnosis and hospitalization (1971 vs. 1732 days, p = 0.84). Arthritis (OR 0.04 (95% CI 0.004–0.35)) and hydroxychloroquine use (OR 0.18 (95% CI 0.04–0.72)) on admission were associated with decreased MAS risk. Admission Systemic Lupus Erythematosus Disease Activity Index scores (30 vs. 19, p = 0.002) and lengths of stay (16 days vs. 3 days, p < 0.0001) were much higher among cases. Death during hospitalization was 19% among cases and 3% among controls ( p = 0.03). Conclusions In this case-control study of hospitalized adult SLE patients, arthritis and hydroxychloroquine use at hospital admission were associated with decreased MAS risk. Further studies are needed to validate these factors associated with lowered MAS risk.

scholarly journals Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Yiming Luo ◽  
Yumeng Wen ◽  
Ana Belen Arevalo Molina ◽  
Punya Dahal ◽  
Lorenz Leuprecht ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Retinal Vasculitis ◽  
Initial Presentation ◽  
Systemic Lupus ◽  
First Case ◽  
Multisystem Involvement ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

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.

Sign in / Sign up

Export Citation Format

Share Document