Abstrakt
Some patients with immunodeficiency develop clinical features of autoimmune disorders. A previously asymptomatic antibody deficiency can underlie the development of autoimmune diseases and a severe course of infection, with a risk of sepsis; such cases are known in selective IgA deficiency.
On the other hand, little information is available on selective IgG subclass deficiencies. An unexpectedly severe course of Campylobacter infection in a 19-year-old-woman with a previously undiagnosed complex immune disorder, including selective IgG1 immunodeficiency, Hashimoto´s autoimmune thyroiditis with hypothyroidism combined with Addison´s disease presumably due to autoimmune adrenalitis, autoimmunity and allergy is described.
The pathophysiological mechanisms of autoimmunity in latent humoral defects are discussed.