AUTO-IMMUNE DISEASES



The human body can sometimes become its own worst enemy.  For reasons still not fully understood the human immune system can lose some of its ability for distinguishing between self and non-self and begin attacking normal healthy cells in the body.  This is a condition known as autoimmune disease.

There are many autoimmune diseases that affect millions of people and their incidence seems to be growing worldwide.

The following Autoimmune Diseases are ones for which intravenous immunoglobulin (IVIg) therapy is indicated and approved:

1. Idiopathic thrombocytopenic purpura (ITP).  Also known as immune thrombocytopenic purpura or autoimmune thrombocytopenic purpura, ITP is an autoimmune bleeding disorder resulting when the immune system attacks its own blood platelets, which are important to the clotting process.  For reasons not well understood, lymphocytes produce antibodies that attach to the platelets, which then do not clot effectively and are subsequently recognized as “foreign” and destroyed in the spleen. Frequent and abnormal bleeding is typical and often results in many small bruises that can look like a rash (purpura).

Children are generally affected with an acute form of the disorder that resolves spontaneously in a few months while in adults it is usually a chronic condition requiring long term treatment.  The disease is rare, with an incidence of 3 cases per 100,000 inhabitants per year in those aged under 16 years and 1.6 to 2.68 cases per 100,000 inhabitants per year in adults, with a slight female preponderance.

Reference (www.kedrion.com):
1. Navarro RP et al.; Considerations for the Optimal Use of Immunoglobulin. Am J Manag Care. 2012;18:S67-S78
2. Abrahamson PE. The incidence of idiopathic thrombocytopenic purpura among adults: a population-based study and literature review. Eur J Haematol..2009 Aug;83(2):83-9.
 

 

2. Kawasaki disease.  Also known as mucocutaneous lymph node syndrome, is a form of vasculitis characterized by inflammation of blood vessels throughout the body. It primarily affects children under the age of five (and rarely over the age of eight).  

With proper treatment the prognosis for these children is good, but without treatment about a quarter of them will develop cardiac problems, including coronary artery aneurysms. Kawasaki disease has become the leading cause of acquired heart disease in children in the developed world.

The cause of Kawasaki Disease is still unknown with researchers divided on whether it is an infection or an autoimmune response, but effective treatment includes primarily intravenous immunoglobulin.

Reference (www.kedrion.com):
1.    Uehara R, Belay ED. “Epidemiology of Kawasaki disease in Asia, Europe, and the United States” J Epidemiol 2012; 22 (2): 79-85
2.    Takahashi K et al.; Pathogenesis of Kawasaki disease. Clinical and Experimental Immunology, 2011; 164 (Suppl. 1): 20–22
3.    Newburger JW. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114:1708–1733