Primary immunodeficiency

2013 ◽  
pp. 1-80
Author(s):  
Gavin P Spickett
Keyword(s):  
B Lymphocyte ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Antibody Deficiency ◽  
Igg Subclass Deficiency ◽  
Selective Iga Deficiency ◽  
Subclass Deficiency ◽  
Bruton’S Disease ◽  
Common Variable

Introduction Classification of immunodeficiency Clinical features of immunodeficiency Investigation of immunodeficiency Laboratory investigation Major B-lymphocyte disorders Rare antibody deficiency syndromes X-linked agammaglobulinaemia (Bruton’s disease) Common variable immunodeficiency (CVID) CVID 2: complications and treatment Selective IgA deficiency IgG subclass deficiency Specific antibody deficiency with normal serum immunoglobulins...

Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency

2007 ◽  
Vol 39 (4) ◽  
pp. 430-431 ◽  
Author(s):  
Emanuela Castigli ◽  
Stephen Wilson ◽  
Lilit Garibyan ◽  
Rima Rachid ◽  
Francisco Bonilla ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Coding Variants ◽  
Common Variable

The Association Between Immunodeficiency and the Development of Autoimmune Disease

1996 ◽  
Vol 10 (1) ◽  
pp. 57-61 ◽  
Author(s):  
J.W. Sleasman
Keyword(s):  
Immune System ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Increased Risk ◽  
High Incidence ◽  
Autoimmune Cytopenias ◽  
The Complement System ◽  
Common Variable

There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of the immune system carry a high risk for the development of autoimmune disease. Inherited deficiencies of the complement system have a high incidence of systemic lupus erythematosus (SLE), glomerulonephritis, and vasculitis. Carrier mothers of children with chronic granulomatous disease, an X-linked defect of phagocytosis, often develop discoid lupus. Several antibody deficiencies are associated with autoimmune disease. Autoimmune cytopenias are commonly observed in individuals with selective IgA deficiency and common variable immune deficiency. Polyarticular arthritis can be seen in children with X-linked agammaglobulinemia. Combined cellular and antibody deficiencies, such as Wiskott-Aldrich syndrome, carry an increased risk for juvenile rheumatoid arthritis and autoimmune hemolytic anemia. Several hypothetical mechanisms have been proposed to explain the associations between autoimmunity and immunodeficiency. Immunologic defects may result in a failure to exclude microbial antigens, resulting in chronic immunologic activation and autoimmune symptoms. There may be shared genetic factors, such as common HLA alleles, which predispose an individual to both autoimmunity and immunodeficiency. Defects within one component of the immune system may alter the way a pathogen induces an immune response and lead to an inflammatory response directed at self-antigens. An understanding of the immunologic defects that contribute to the development of autoimmunity will provide an insight into the pathogenesis of the autoimmune process.

scholarly journals Linkage of IgA deficiency to Gm allotypes; the influence of Gm allotypes on IgA-IgG subclass deficiency

2008 ◽  
Vol 99 (2) ◽  
pp. 211-215 ◽  
Author(s):  
V.-A. OXELIUS ◽  
A.-M. CARLSSON ◽  
L. HAMMARSTRÖM ◽  
J. BJÖRKANDER ◽  
L. Å. HANSON
Keyword(s):  
Iga Deficiency ◽  
Igg Subclass ◽  
Igg Subclass Deficiency ◽  
Gm Allotypes ◽  
Subclass Deficiency

scholarly journals Salmonella typhi vaccination response study reveals defective antibody production selective IgA deficiency patient

2015 ◽  
Vol 62 (4) ◽  
pp. 318-322
Author(s):  
Daniel E Pleguezuelo ◽  
Carla Gianelli
Keyword(s):  
Antibody Response ◽  
Antibody Production ◽  
Tetanus Toxoid ◽  
Common Variable Immunodeficiency ◽  
Salmonella Typhi ◽  
Iga Deficiency ◽  
Upper Respiratory Tract ◽  
Selective Iga Deficiency ◽  
Tract Infections ◽  
Common Variable

Selective IgA deficiency (SIgAD) is the most prevalent immunodeficiency worldwide, progressing to common variable immunodeficiency only in few reported cases. We report the case of a Spanish female aged 22 and diagnosed of selective IgA deficiency, a long history of bronchitis, several episodes of pneumonia, bilateral bronchiectasis, normal IgG, IgM, IgG subclasses, and detectable pre-vaccination IgG antibodies against tetanus toxoid and Streptococcus pneumoniae. She was evaluated in our clinic in order to rule out common variable immunodeficiency. We observed good antibody response to tetanus toxoid, absence of circulating switched memory B cells, decreased response to pneumococcal polysaccharide antigens and a lack of response to Salmonella typhi vaccine. Most SIgAD patients presents with upper respiratory tract infections or mild diarrhea. Those with lower tract infections, pneumonia or untreatable diarrhea should follow B-cell subpopulations’ study and antibody response to vaccines. Absence of response to Salmonella typhi vaccine allowed us to expose the defective antibody production.

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