General Approach and Clinical Management for Adults with Primary Immunodeficiency Diseases

immunoloji-17-1-2024

Mustafa İlker İNANa , Özgür KARTALa
aUniversity of Health Sciences Gülhane Training and Research Hospital, Clinic of Immunology and Allergy Diseases, Ankara, Türkiye

İnan Mİ, Kartal Ö. General approach and clinical management for adults with primary immunodeficiency diseases. Çölkesen F, ed. Primary Immunodeficiency Diseases in Adults. 1st ed. Ankara: Türkiye Klinikleri; 2024. p.1-9.

ABSTRACT
Primary immunodeficiency diseases (PIDs), recently called inborn errors of immunity, are a growing group of diseases. Increased susceptibility to all types of infections is the predominant clinic of all PIDs. However, a significant number of patients may also present with the manifestation of allergic diseases, autoimmune/autoinflammatory diseases and lymphoproliferative/malignant diseases without a history of infection. Immunoglobulin replacement therapy (IgRT) is the cornerstone of the treatment. Although it was shown that IgRT significantly reduces the infection rate and increases patient’s survival, it seems to be failed in controlling non-infectious complications. Thus, these complications should be actively investigated as a part of the diagnosis, patient phenotyping and in the course of the disease. Also a careful long-term follow-up is necessary for the management of complications. After the increased usage of genetic tests which are helping the understanding of underlying cause, new targeted treatment modalities are progressing and giving hope for a better prognosis.

Keywords: Primary immunodeficiency diseases; immunoglobulins, intravenous; complications; autoimmunity

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