853 Autoimmune Disease in Children with Deficit in Immunoglobulin A

Introduction and objectives: The immunodeficiencies (ID), by the subsequent impairing of the immunoregulation, may be at the origin of certain autoimmune diseases (AID). The relationship between the ID and the AID is triggered by opportunistic infections, the latter favorised by the underlying ID. The deficit in immunoglobulin A is one of the most frequent ID associated with AID. Material: We realized a prospective study on 87 patients diagnosed with AID. We analyzed the initial immune status, for all these patients. For another group of 53 children, diagnosed with selective IgA immunodeficiency, we realized a six year period survey of the level of the T suppressor lymphocytes, the T helper/ T suppressor ratio, and of the presence of auto antibodies: Anti DNA, rheumatoid factor (RF). Results: 8 patients (8%), from the group diagnosed with AID, were also identified with selective deficit in IgA at the moment of the initial diagnosis. In the group of 53 patients with underlying IgA immunodeficiency, 2 patients developed over the 6 years of the survey, a significative titer of anti DNA antibodies. In one patient the presence of the RF was detected, 4 children presented a decrease of the T suppressor level, with a rise of the immune ratio. None of these patients presented clinical signs suggesting an AID. Conclusions: The Ig A immunodeficiency may be a risk factor for subsequent AID. There is a higher risk for AID in patients who develop anti DNA antibodies, RF or a persistent decrease of the T suppressor lymphocytes.