TY - JOUR
T1 - Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes
AU - Agarwal, Shradha
AU - Mayer, Lloyd
PY - 2009/10
Y1 - 2009/10
N2 - Primary humoral immune deficiencies are characterized by limited antibody responses secondary to either impaired B-lymphocyte development or B-cell responses to T-lymphocyte signals. Given that the gastrointestinal tract is the largest lymphoid organ in the body, it is not surprising that intestinal diseases are common in immunodeficiency. These gastrointestinal diseases can be classified into one of 4 groups, infection, malignancy, inflammatory, and autoimmune, and can mimic other known disease processes, such as inflammatory bowel disease and celiac sprue. The exact pathogenesis of these gastrointestinal disorders in the setting of systemic immunodeficiency is still under investigation. However, studies suggest that defects in antibody deficiency alone do not result in gastrointestinal disease but rather that defects in cellular immunity are also involved. Treatment is difficult given an already immunocompromised state, and often therapy with immunomodulators is required for more severe processes.
AB - Primary humoral immune deficiencies are characterized by limited antibody responses secondary to either impaired B-lymphocyte development or B-cell responses to T-lymphocyte signals. Given that the gastrointestinal tract is the largest lymphoid organ in the body, it is not surprising that intestinal diseases are common in immunodeficiency. These gastrointestinal diseases can be classified into one of 4 groups, infection, malignancy, inflammatory, and autoimmune, and can mimic other known disease processes, such as inflammatory bowel disease and celiac sprue. The exact pathogenesis of these gastrointestinal disorders in the setting of systemic immunodeficiency is still under investigation. However, studies suggest that defects in antibody deficiency alone do not result in gastrointestinal disease but rather that defects in cellular immunity are also involved. Treatment is difficult given an already immunocompromised state, and often therapy with immunomodulators is required for more severe processes.
KW - Immunodeficiency
KW - gastrointestinal disease
KW - humoral immunodeficiency
KW - inflammatory intestinal disease
UR - http://www.scopus.com/inward/record.url?scp=70449713437&partnerID=8YFLogxK
U2 - 10.1016/j.jaci.2009.06.018
DO - 10.1016/j.jaci.2009.06.018
M3 - Article
C2 - 19665769
AN - SCOPUS:70449713437
SN - 0091-6749
VL - 124
SP - 658
EP - 664
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
IS - 4
ER -