Master Class on Primary Immunodeficiencies: 7-8 April 2017, Prague, The Czech Republic
The 18th Biennial Meeting of ESID will be held in Lisbon, Portugal. 24 - 27 October 2018
In the last few years novel developments in gene discovery and increased knowledge in the mechanisms that govern immune system functions have resulted in the identification of several novel primary immunodeficiency (PID) syndromes. Since years it is known that several forms of primary immunodeficiencies, mostly those associated with dysregulation of immune reactions, may present with allergic reactions and/or show markers and/or symptoms that are typically connected to allergy, such as the presence of high IgE levels, the predominance of Th2 immune response, allergic or allergic-like inflammatory events including skin reactions that may mimick allergic reactions. As an example, Omenn’s syndrome, Hyper IgE syndromes or IPEX are characterized by frequent allergic manifestations, that include eczema, allergies to specific foods, and more rarely asthma.
Studies of mechanisms that favor allergic phenomena in the context of PID can help to shed mechanistic lights on pathways that are involved the pathogenesis of atopy and allergic diseases. Allergic disorders, in general, are not monogenic and their pathogenesis is, from immunological point of view, complex. Such pathogenesis, however, includes immune reactions that, as stated above, have been in depth defined in primary immunodeficiency field. Hopefully, understanding the effect of disruptions of these pathways and the comprehension of the the relationship between the selective, primary immune defects and the development of allergy could provide insight into the diagnosis and clinical management of the more common allergic diseases
The Task Force proposed by our PID Interest Group is aimed to identify and clarify the presence of pathogenetic connections between the immune defect that characterizes some primary immunodeficiencies and the development of allergies, via the following step:
Guidelines for diagnosis of PIDs, specifically immunodeficiencies co-occurring with allergies
As a first step we plan to aim at the clinical aspects of such overlapping field and we want to create clear guidelines for diagnosis of primary immunodeficiencies underlying and/or complicating some allergy presentations (such as hypogammaglobulinemia associated with atopic dermatitis and/or respiratory allergy in pediatric age, diagnosis of hyper IgE syndrome versus severe atopy, fast diagnosis of severe immunodeficiencies with dominant allergy features such as Omenn syndrome e.t.c). For that we´ll introduce to allergologists 10 warning signs for immunodeficiency, and we´ll modify diagnostic criteria for PIDs with regard to allergic clinical presentation.
Guidelines for laboratory investigation in order to verify immune mechanisms behind allergy-primary immunodeficiency
Subsequently we plan to review and clearly present a set of laboratory parameters useful for elucidating a role of potential immunodeficiency in a development of allergy. Striking example of such situation is an unrecognized antibody deficiency complicating a course of allergic disease and defects of T regulatory cells in primary immunodeficiencies.
Education of allergologists about PID field, increase awareness about PIDs
As a next step we plan to educate a wide field of allergy specialists about treatments of immunodeficiencies associated with allergies. In most of those states some form immunoglobulin therapy might have a rational indication and might improve a long-term treatment of allergy.