Featured Research

from universities, journals, and other organizations

Macrophage activation syndrome: Clues to calming a cytokine storm

Date:
May 17, 2011
Source:
Children's Hospital of Philadelphia
Summary:
By analyzing complex interactions of the immune system in an animal study, pediatric researchers have found potential tools for controlling a life-threatening condition called a cytokine storm that may strike children who have juvenile arthritis and other autoimmune diseases. The study is a first step toward developing new treatments for a condition called macrophage activation syndrome.

By analyzing complex interactions of the immune system in an animal study, pediatric researchers have found potential tools for controlling a life-threatening condition called a cytokine storm that may strike children who have juvenile arthritis and other autoimmune diseases.

Related Articles


In a cytokine storm, the body's immune system rages out of control, resulting in overwhelming inflammation, rapid organ failure and death if not quickly diagnosed and treated. In addition to children with juvenile arthritis, patients with lupus or Epstein-Barr virus infection may also suffer this complication, called macrophage activation syndrome (MAS).

"Our study is a first step toward developing new treatments for MAS," said study leader Edward M. Behrens, M.D., a pediatric rheumatologist at The Children's Hospital of Philadelphia. The researchers published their study online May 16 in the Journal of Clinical Investigation.

Because MAS occurs rarely in children, it is difficult to investigate in patients. Therefore, animal studies are important, said Behrens, whose team was able to develop a variety of mice into the first animal model of MAS. Their study also demonstrated the mechanism of MAS differs importantly from another disease that manifests similar symptoms.

That other disease, called HLH (for hemophagocytic lymphohistiocytosis), also causes a life-threatening cytokine storm in children. Because of the similar manifestations, he added, physicians may use similar treatments for both HLH and MAS -- but this is not always appropriate.

HLH is caused by a genetic mutation, but MAS is not. Instead, Behrens and colleagues were able to explain how inflammation from rheumatological diseases like systemic juvenile arthritis causes MAS by acting through immunological pathways. In particular, Behrens showed that overactive immune system proteins called Toll-like receptors interact with the immune system to drive MAS in the absence of gene mutations or infectious triggers.

"We identified two important molecules in the immune system that control the severity of MAS," said Behrens. One molecule is interferon-gamma, which makes MAS more severe. The other molecule is interleukin-10 (IL-10), which has a protective effect. "This research strongly suggests that the relative contribution of these molecules can dial up or dial down the severity of a cytokine storm in MAS," said Behrens.

"Our next studies will be to investigate whether we can reduce the action of interferon-gamma, or enhance the beneficial effects of IL-10, as possible treatments for children who experience this syndrome," Behrens added.

Support for this work came from the National Institutes of Health, an Arthritis Foundation Innovative Research Grant, and a Howard Hughes Medical Institute Early Career Physician Scientist Award to Behrens.


Story Source:

The above story is based on materials provided by Children's Hospital of Philadelphia. Note: Materials may be edited for content and length.


Journal Reference:

  1. Edward M. Behrens, Scott W. Canna, Katharine Slade, Sheila Rao, Portia A. Kreiger, Michele Paessler, Taku Kambayashi, Gary A. Koretzky. Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice. Journal of Clinical Investigation, 2011; DOI: 10.1172/JCI43157

Cite This Page:

Children's Hospital of Philadelphia. "Macrophage activation syndrome: Clues to calming a cytokine storm." ScienceDaily. ScienceDaily, 17 May 2011. <www.sciencedaily.com/releases/2011/05/110516121425.htm>.
Children's Hospital of Philadelphia. (2011, May 17). Macrophage activation syndrome: Clues to calming a cytokine storm. ScienceDaily. Retrieved March 5, 2015 from www.sciencedaily.com/releases/2011/05/110516121425.htm
Children's Hospital of Philadelphia. "Macrophage activation syndrome: Clues to calming a cytokine storm." ScienceDaily. www.sciencedaily.com/releases/2011/05/110516121425.htm (accessed March 5, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, March 5, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Bupa Eyes India Healthcare Opportunities

Bupa Eyes India Healthcare Opportunities

Reuters - Business Video Online (Mar. 5, 2015) — Bupa is hoping to expand in India&apos;s fast-growing health insurance market, once a rule change on foreign investment is implemented. The British private healthcare group&apos;s CEO tells Grace Pascoe why it&apos;s so keen on the new opportunity. Video provided by Reuters
Powered by NewsLook.com
Doctor in Your Pocket Is Getting Smarter

Doctor in Your Pocket Is Getting Smarter

Reuters - Business Video Online (Mar. 5, 2015) — Mobile apps are turning smartphones into a personal doctors, with users able to measure heart rate, blood pressure and even blood sugar. But will it change our behaviour? Ivor Bennett reports from the Mobile World Congress in Barcelona. Video provided by Reuters
Powered by NewsLook.com
AbbVie Inks $21B Deal To Buy Cancer Drugmaker Pharmacyclics

AbbVie Inks $21B Deal To Buy Cancer Drugmaker Pharmacyclics

Newsy (Mar. 5, 2015) — AbbVie announced Wednesday it will buy cancer drugmaker Pharmacyclics in a $21 billion deal. Video provided by Newsy
Powered by NewsLook.com
Adults Only Get The Flu Twice A Decade, Researchers Say

Adults Only Get The Flu Twice A Decade, Researchers Say

Newsy (Mar. 4, 2015) — Researchers found adults only get the flu about once every five years. Scientists analyzed how a person&apos;s immunity builds up over time as well. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins