Hereditary angioedema is an autosomal dominant disorder of the complement system in which there is a deficiency of the inhibitor of the activated first component of complement. We have previously reported on three generations of a family with classic hereditary angioedema. Three members of this family have now developed IgA nephropathy. The association of hereditary angioedema with various immu...

Despite our knowledge on the role of IgA in mucosal homeostasis and host defense and clinical evidence suggesting deficient first-line defense mechanisms in chronic airway disorders, little is known regarding its role in asthma and chronic obstructive pulmonary disease (COPD). Studies suggest that the mucosal IgA response is impaired in COPD, and a deficient transport of IgA across the bronchia...

Immunoglobulin A (IgA) secretion by plasma cells in the immune system is critical for protecting the host from environmental and microbial infections. However, the molecular mechanisms underlying the generation of IgA(+) plasma cells remain poorly understood. Here, we report that the B cell-expressed tetraspanin CD37 inhibits IgA immune responses in vivo. CD37-deficient (CD37-/-) mice exhibit a...

Abstract The microbiome has emerged as a contributor to type 1 diabetes (T1D), but how interactions between the and mucosal immune system lead or prevent T1D is not clear. Previously, we found that deletion of B cell signaling protein Bruton’s Tyrosine Kinase (BTK) selectively eliminates autoreactive cells autoantibodies, preventing development. However, discovered this disease protection micro...

BACKGROUND The prevalence of primary immunodeficiency (PID) in the relatives of patients with common variable immunodeficiency (CVID) and IgA deficiency is high. Allergic disorders have been recorded in patients with humoral immunodeficiency. We aimed to determine the frequency of humoral immunodeficiency and atopy in the relatives of patients with CVID. METHODS The study population comprised...

Celiac disease has been associated with selective IgA immunoglobulin deficiency. A celiac disease cohort of 234 biopsy-defined adults (including 73 males and 161 females) from a 30-year period from 1982 to 2011 was reviewed. A total of 7 with selective IgA immunoglobulin deficiency were noted, or about 3%. All were female with an initial positive biopsy for untreated celiac disease. All had cha...

background: in some studies inflammatory bowel disease (ibd) and celiac disease were considered to be associated and some belive that this association may influence the prognosis of ibd. however, there is a cosiderable controversy regarding this association. therefore ,we aimed to assess the association of these two common digestive diseases and evaluate the complications of this association. m...