Descriptive review of clinical data from 186 records of outpatients with IgA deficiency accompanied at a quaternary hospital in Brazil

Abstract

IgA Deficiency (IgAD) is the most common immunoglobulin deficiency, with an approximate incidence of 1 in 400 to 3000 individuals in the general population. Selective IgA deficiency is defined as serum IgA levels less than 7 mg/dL with normal serum IgM and IgG levels. A subgroup of individuals, with more than 7 mg/dL but less than 30 mg/dL of serum IgA, are defined as partial IgA deficient patients (pIgAD). Many are clinically asymptomatic, however some patients have increased incidence of infections, as well as presence of atopy, autoimmunity and cancer. Our objective was to report clinical data observed in a cohort of patients followed between 1994 and 2014. The most common associated condition found was atopic disease in about 60% of the patients, specially rhinitis (in t IgAD and pI gAD). The recurrent infection prevalence was 36%: 20% of sinusitis, otitis and amigdalitis, 10% of recurrent pneumonia and 18% of recurrent diarrhea. Autoimmunity condition was observed in 18%of the patients, like thyroiditis ( the most prevalent, 13%), autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, rheumatoid arthritis, celiac-like disease, systemic lupus erythematosus, Sjögren's disease, juvenile rheumatoid arthritis, autoimmune hepatitis and vitiligo; 9% of them had history of medication reaction and 8% had contact dermatitis. About 9% of the IgAD patients had malignancies or pre neoplasic conditions likeMGUS. The exact value of deaths among these patients is difficult to calculate because many patients lose up for unknown reasons, but so far only one death was reported, and was attributed to sepsis.