Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers

2017 ◽  
Vol 16 (1) ◽  
pp. 16-21 ◽  
Author(s):  
Piero Ruscitti ◽  
Paola Cipriani ◽  
Francesco Ciccia ◽  
Francesco Masedu ◽  
Vasiliki Liakouli ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Autoimmune Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Patients ◽  
Disease Analysis ◽  
Activation Syndrome

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

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.

scholarly journals Etoposide as an Effective Drug for Adult Macrophage Activation Syndrome: A Retrospective Study

2021 ◽  
Author(s):  
Lingbo He ◽  
Zhili Jin ◽  
Menghan Liu ◽  
Tingting Cui ◽  
Lin Wu ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Initial Treatment ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Hemophagocytic Syndrome ◽  
Response Rate ◽  
Initial Therapy ◽  
Group 2 ◽  
Activation Syndrome ◽  
Group 1

Abstract BackgroundAutoimmune disease related hemophagocytic syndrome, in other words, macrophage activation syndrome(MAS), is a rare, but lethal complication of autoimmune disease. At present, specific treatment guidelines for adult MAS have not been formulated, most experience are derived from children, researches about etoposide are scarce. As the importance of etoposide in the initial treatment had been proved in other subtypes of hemophagocytic syndrome, the objective of this study is to investigate the effectiveness of etoposide in the treatment of the adult macrophage activation syndrome.Result74 patients with autoimmune disease related hemophagocytic syndrome were involved in this study, they were divided into two groups based on initial treatment, group 1(n=53): initial therapy did not contain etoposide, group 2(n=21): initial therapy contained etoposide. The overall response rate and complete response rate of group 2 were significantly higher than group 1(ORR 90.5% vs 24.5%, CRR 33.3% vs 3.8%, P<0.05). Patients with different HLH remission states have significantly different prognosis(P<0.001).ConclusionAdopting VP-16 in initial treatment can significantly increase the OR rate and CR rate of adult MAS patients, and the HLH states influenced the prognosis significantly.

scholarly journals Macrophage Activation Syndrome in Autoimmune Disease

2010 ◽  
Vol 153 (2) ◽  
pp. 109-120 ◽  
Author(s):  
Sean Deane ◽  
Carlo Selmi ◽  
Suzanne S. Teuber ◽  
M. Eric Gershwin
Keyword(s):  
Autoimmune Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activation Syndrome

scholarly journals Macrophage activation syndrome in a newborn infant born to a mother with autoimmune disease

2015 ◽  
Vol 35 (2) ◽  
pp. 158-160 ◽  
Author(s):  
J H Park ◽  
S H Kim ◽  
H J Kim ◽  
S J Lee ◽  
D C Jeong ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Newborn Infant ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activation Syndrome
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