Systemic Arthritis

Systemic Arthritis:

• Affects the entire body, the joints, causes fevers, and a faint rash.
•  Affects only 10% to 15% of children and teenagers with Juvenile Arthritis.
•  A very severe form of Arthritis 
• If the disease is severe, a child or teenager with systemic arthritis may have inflammation around her heart, lungs, or bowels. Also, the immune system may start to destroy blood cells.
• Auto Immune disease.

The Defect in the signaling pathway: Notch Pathway

Notch pathway influenced the development of macro phages, a type of immune cell that usually attacks invading pathogens. When macro phage development goes wrong, however, the cells can cause widespread inflammation and destruction — attacking joints and contributing to  arthritis. The defect in the Notch Pathway is what causes arthritis.A cell receives signals from its neighbors through the Notch pathway, and responds to such signals with certain behavior changes, such as regulation of its own gene expression. The pathway affected the macro phage differentiation is what causes the pathway to respond wrongly causing a defect and creating arthritis in the body.

What this disease does to the joints

How Sulfasalazine blocks a signaling pathway

• A pill given twice a day
• A sulfa drug
• Given to arthritis patients 
•  anti-inflammatory drug
• Reduces inflammation inside the body

 NF-κB pathway comprises a family of inducible transcription factors that serve as important regulators of the host immune and inflammatory response. In addition, NF-κB pathway also involved in protecting cells from undergoing apoptosis in response to DNA damage or cytokine treatment. Stimulation of the NF-κB pathway is mediated by diverse signal transduction cascades. These signals activate the IκB kinases, IKKα and IKKβ, which phosphorylate inhibitory proteins known as IκB to result in their ubiquitination and degradation by the proteasome. The degradation of IκB results in the translocation of NF-κB from the cytoplasm to the nucleus where it activates the expression of specific cellular genes. As we better understand the regulation of the NF-κB pathway, the potential for inhibiting this pathway has received attention. Sulfasalazine inhibits this pathway and can reduce the inflammatory response in arthritis patients.

What Sulfusalazine looks like

 

Websites Used:

• http://www.aboutkidshealth.ca/en/resourcecentres/juvenileidiopathicarthritis/aboutjia/typesofjiainchildrenandteenagers/pages/systemicarthritis.aspx
• http://healthland.time.com/2012/05/25/rheumatoid-arthritis-scientists-discover-a-cell-signaling-pathway-that-may-lead-to-new-treatment/
• http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682204.html
• http://www.jci.org/articles/view/11914
• Google Images