The Study Group was established by the Italian Society of Allergy and Clinical Immunology for the preparation of guidelines regarding various forms of primitive immunodeficiency. In this study such guidelines are reported for the diagnosis and therapy of Common Variable Immunodeficiency (CVID), according to a group of experts composed of clinical immunologists, infective disease specialists, nutritionists, molecular biologists and gene therapists. CVID represents a heterogenous group of various symptoms which generally manifest themselves in adolescence or in adulthood. Symptoms include recurrent respiratory and gastroenteric infections which if not treated early on can lead to chronic respiratory or gastroenteric insufficiency, bronchiectasis, chronic diarrhoea and malnutrition. In a part of subjects, autoimmune diseases can develop such as idiopathic trombocytopenic purpura, autoimmune haemolytic anemia, gastroenteric neoplasia or lymphoma. 10% of subjects will also face a chronic disease such as non caseosis granulomatosis of lymphnodes or spleen. In only 5% of patients a molecular genetic defect has been found, related mainly to the absence or severe defect of regolatory molecules of immune response in B or T cells. A severe diminution of Ig classes or subclasses is relevant for the diagnosis. In addition a defect of CD4 cells has been recently reported in almost 50% of patients. The diminution of naive T cells is present mainly in CVID patients with severe recurrent infection. The authors prepared guidelines for both the prophylaxis and therapy of infections as well as the correct substitution therapy with i.v. IgG. In addition, social and psychological assistance factors are reported since they are crucial in the correct clinical maintenance of patients. Prospects for the future are also mentioned concerning the improvement of molecular diagnosis and identification of new therapeutic strategies.
Italian Society Of Allergy And Clinical Immunology Guidelines For The Diagnosis And Treatment Of Common Variable Immunodeficiency (Cvid) / Aiuti, Fernando; A., Aiuti; L., Calza; F., Chiodo; W., DE SANTIS; Dettorre, G.; L., Emmi; A., Isgro'; Luzi, Giuseppe; E., Maggi; M., Marziali; Mezzaroma, Ivano; M., Montroni; Muscaritoli, Maurizio; R., Paganelli; F., Pandolfi; S., Starnino; Sirianni, Maria Caterina; Vullo, Vincenzo. - In: GIORNALE ITALIANO DI ALLERGOLOGIA E IMMUNOLOGIA CLINICA. - ISSN 1120-6373. - STAMPA. - 16 (S1):(2006), pp. 1-30.
Italian Society Of Allergy And Clinical Immunology Guidelines For The Diagnosis And Treatment Of Common Variable Immunodeficiency (Cvid)
AIUTI, Fernando;G. DETTORRE;LUZI, Giuseppe;MEZZAROMA, Ivano;MUSCARITOLI, Maurizio;SIRIANNI, Maria Caterina;VULLO, Vincenzo
2006
Abstract
The Study Group was established by the Italian Society of Allergy and Clinical Immunology for the preparation of guidelines regarding various forms of primitive immunodeficiency. In this study such guidelines are reported for the diagnosis and therapy of Common Variable Immunodeficiency (CVID), according to a group of experts composed of clinical immunologists, infective disease specialists, nutritionists, molecular biologists and gene therapists. CVID represents a heterogenous group of various symptoms which generally manifest themselves in adolescence or in adulthood. Symptoms include recurrent respiratory and gastroenteric infections which if not treated early on can lead to chronic respiratory or gastroenteric insufficiency, bronchiectasis, chronic diarrhoea and malnutrition. In a part of subjects, autoimmune diseases can develop such as idiopathic trombocytopenic purpura, autoimmune haemolytic anemia, gastroenteric neoplasia or lymphoma. 10% of subjects will also face a chronic disease such as non caseosis granulomatosis of lymphnodes or spleen. In only 5% of patients a molecular genetic defect has been found, related mainly to the absence or severe defect of regolatory molecules of immune response in B or T cells. A severe diminution of Ig classes or subclasses is relevant for the diagnosis. In addition a defect of CD4 cells has been recently reported in almost 50% of patients. The diminution of naive T cells is present mainly in CVID patients with severe recurrent infection. The authors prepared guidelines for both the prophylaxis and therapy of infections as well as the correct substitution therapy with i.v. IgG. In addition, social and psychological assistance factors are reported since they are crucial in the correct clinical maintenance of patients. Prospects for the future are also mentioned concerning the improvement of molecular diagnosis and identification of new therapeutic strategies.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
