Common variable immunodeficiency - an update
نویسندگان
چکیده
Common variable immunodeficiency (CVID) describes a heterogeneous subset of hypogammaglobulinemias of unknown etiology. Typically, patients present with recurrent bacterial infections of the respiratory and gastrointestinal tract. A significant proportion of CVID patients develops additional autoimmune, inflammatory or lymphoproliferative complications. CVID is the most frequent symptomatic primary immunodeficiency encountered in adults. Informative monogenetic defects have been found in single patients and families but in most cases the pathogenesis is still elusive. Numerous immunological studies have demonstrated phenotypic and functional abnormalities of T cells, B cells and antigen-presenting cells. A hallmark is the impaired memory B-cell formation that has been taken advantage of for classifying CVID patients. Clinical multi-center studies have demonstrated a correlation between immunological markers and clinical presentation. Long-term outcome is significantly influenced by delay of diagnosis and treatment and the presence of chronic inflammatory complications. While immunoglobulin replacement therapy plus antibiotics can control infections in most cases, patients with non-infectious inflammatory complications such as granulomatous inflammation, interstitial lung disease, inflammatory bowel disease, lymphoproliferation and developing malignancies still represent a therapeutic challenge. In this review we provide a systematic overview of the immunological, clinical, diagnostic and therapeutic aspects of CVID and highlight recent developments in these fields.
منابع مشابه
A 20-YEAR SURVEY OF INFECTIOUS COMPLICATIONS IN 64 PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY
Common variable immunodeficiency (CV ID) is a heterogeneous primary immunodeficiency disorder, characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. To determine the spectrum of infectious complications in patients with common variable immunodeficiency (CV ID), we reviewed the hospital records of 64 CVID patients, who were diagnosed in Child...
متن کاملCo-existence of Common Variable Immunodeficiency (CVID) with Idiopathic Thrombocytopenic purpura (ITP)
متن کامل
A CASE REPORT OF COMMON VARIABLE IMMUNODEFICIENCY(CV ID) WITH LEUKOCYTES ADHESION DISORDERS (LADS)
A three year old boy with delayed separation of the umbilical cord, recurrent pyogenic infections and common variable immundeficiency is presented. The immunoglobulin measurement was severely abnormal. To the best of our knowledge, the existance of leukocyte adhesion disorder together with common variable immunodeficiency (CYID) in a patient has not been reported prior to this study.
متن کاملIVIG Anaphylaxis in a patient with Common variable immune deficiency disease (CVID).
Common variable immune deficiency disease (CVID) is one of the most common primary immunodeficiency disorders. It causes reduction of immunoglobulin levels and specific antibodies production and enhancement of recurrent and chronic infections risk, especially respiratory infections and gastrointestinal tracts. Here, we report a case of CVID in a 3 years old male with recurrent hospitalizations ...
متن کاملA Review of Primary Immunodeficiency Diseases with Skin Manifestations
Introduction: Primary immunodeficiencies (PID) are rare heterogeneous disorders with defects in which one or more components of the immune system are malfunctioning. Clinical presentations of the patients according to type of immunodeficiency are variable. The majority of these patients are susceptible to infections depending on the type of disorder. In these patients, one of the most important...
متن کاملعوارض ناشی از تزریق ایمونوگلوبولین وریدی در بیماران با نقص ایمنی اولیه
Background and Purpose: Intravenous immunoglobulin is used for primary immunodeficiency disorders. There have been some reports that intravenous immunoglobulin causes side effects. The aim of this study was to investigate intravenous immunoglobulin side effects in immunodeficiency patients. Materials and Methods: The study utilized the data of 29 primary immunodeficiency patients that were...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 14 شماره
صفحات -
تاریخ انتشار 2012