Клиническое наблюдение пациента с хроническим риносинуситом и редкой формой первичного иммунодефицита

Abstract

The article tentatively reviewed such rare immuno-dependent conditions as: IgG4-linked primary immunodeficiency (PI) and general variable immunodeficiency. Over the past two decades, more and more data has been accumulating about this subject, due to which several previously known nosological forms were combined into a new group of IgG4-associated diseases (IgG4-SZ), and in order to establish a diagnosis of IgG4-related systemic disease, two or more lesions of organs are required. This group of diseases has a common serological sign – an increase in the concentration of IgG4 subclass in serum. Histologically in organs and tissues, plasmocytes infiltration secreting IgG4, eosinophils are formed, fibrosclerosis and obliterating phlebitis develop. Laboratory and immunological examination of patients with PI helps to identify a specific violation of the immune system. Primary diagnosis is usually done using a panel of screening laboratory tests. A high risk of developing IgG4-C3 is in patients with pancreatitis with unknown etiology, sclerosing cholangitis, bilateral enlargement of the salivary and lacrimal glands, in the presence of multiple nodes in the lungs. Predisposing factors are middle age and male gender. The article presents a clinical observation of a patient suffering from a rare combination of primary immunodeficiency and chronic bilateral rhinosinusitis, special attention on laboratory and instrumental examinations and treatment.