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Impact of intervenous immunoglobulin (IVIG) treatment among patients with Primary Immunodeficiency diseases

Primary immunodeficiency diseases (PIDD) represent a class of disorders in which there is an intrinsic defect in the human immune system (rather than immune disorders that are secondary to infection, chemotherapy, or some other external agent). In some cases, the body fails to produce any or enough antibodies to fight infection. In other cases, the cellular defenses against infection fail to work properly. There are more than 150 different primary immunodeficiency diseases currently recognized by the World Health Organization [1]. Primary immunodeficiency diseases are often described as rare disorders, but the worldwide incidence and population prevalence of these diseases, either individually or in the aggregate, is not well established. There have been some estimates of the incidence and prevalence of some disorders based on primary immunodeficiency registries in various countries [2,3]. In 2005, a national telephone survey of 10,000 households by the Immune Deficiency Foundation (IDF) yielded an estimated population prevalence of diagnosed primary immunodeficiency diseases at 1 in 1,200 persons in the United States. When applied to the U.S. population of 297,386,000 persons, this suggests approximately 250,000 persons have a diagnosed primary immunodeficiency disease in the United States [4]. Unfortunately no similar study has been done in Europe. The Immune Deficiency Foundation has commissioned a series of surveys over the past decade to try to estimate the characteristics and experiences of patients with primary immunodeficiency diseases in the United States. The First National Patient Survey was conducted by IDF in 1996–1997. Nearly 3,000 persons with a diagnosis of a primary immunodeficiency disease participated in this survey. Those patients