The paper presents data on the pathogenesis of systemic lupus erythematosus (SLE), and depicts various molecular mechanisms for the development of SLE and lupus-like syndromes. It describes groups of diseases, such as apoptotic defects; NETosis; interferonopathies; complement deficiency; autotolerance disorders associated with mutations in the RAG1/RAG2 genes; hereditary metabolic diseases (prolidase deficiency, deficiency of adenosine deaminase 2; lysinuric protein intolerance; and α-mannosidase deficiency). The table summarizes clinical data on most of the known lupus-like syndromes and their molecular mechanisms.The pathogenesis of many forms of monogenic lupus-like diseases is being studied. The main sign suggesting in favor of the possible monogenic disease in a patient with SLE is its onset in infancy, especially in males. Attention should be also paid to a compromised family history, including to the marriage between close relatives, the resistance of disease to standard therapy, as well as atypical symptoms.

Malignant tumors are often found in patients with hereditary defects of immunity and are one of the main causes of death of such patients. The increased risk of developing tumors appears to be due to the weakening of antitumor surveillance and vulnerability to oncogenic viruses. The presence of primary immunodeficiency in a patient with an oncological disease can affect the tactics of treatment. Expanding the possibilities of primary immunodeficiency therapy dictates the need for timely differential diagnosis of hereditary immunodeficiencies.

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