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.

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 September 23, 2014 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 September 23, 2014).

Share This



More Health & Medicine News

Tuesday, September 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola Costs Keep Mounting

Ebola Costs Keep Mounting

Reuters - Business Video Online (Sep. 23, 2014) The WHO has warned up to 20,000 people could be infected with Ebola over the next few weeks. As Sonia Legg reports, the implications for the West African countries suffering from the disease are huge. Video provided by Reuters
Powered by NewsLook.com
Ebola Cases Could Reach 1.4 Million Within 4 Months

Ebola Cases Could Reach 1.4 Million Within 4 Months

Newsy (Sep. 23, 2014) Health officials warn that without further intervention, the number of Ebola cases in Liberia and Sierra Leone could reach 1.4 million by January. Video provided by Newsy
Powered by NewsLook.com
WHO: Ebola Cases to Triple in Weeks Without Drastic Action

WHO: Ebola Cases to Triple in Weeks Without Drastic Action

AFP (Sep. 23, 2014) The number of Ebola infections will triple to 20,000 by November, soaring by thousands every week if efforts to stop the outbreak are not stepped up radically, the WHO warned in a study on Tuesday. Duration: 01:01 Video provided by AFP
Powered by NewsLook.com
5 Ways Men Can Prevent Most Heart Attacks

5 Ways Men Can Prevent Most Heart Attacks

Newsy (Sep. 23, 2014) No surprise here: A recent study says men can reduce their risk of heart attack by maintaining a healthy lifestyle, which includes daily exercise. 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