Primary immunodeficiency, also termed as inborn errors of immunity(IEI), is characterized by not only susceptibility to infection but also malignancy, allergy, autoinflammation, and autoimmunity. Most of the patients with IEI manifest susceptibility to bacterial and viral infections. The mainstay of treatment for IEI includes 1)administration of antibiotics and prevention of bacterial, fungal, and viral infections and 2)immunoglobulin replacement therapy(IgRT)for antibody deficiency. The drug combination sulfamethoxazole-trimethoprim(ST)is used in a majority of patients with IEI to prevent infections. It is generally administered three times a week for the prevention of
Pneumocystis pneumonia, whereas it must be administered daily for the prevention of bacterial infection. Chronic granulomatous disease is a phagocytic disorder and is sometimes associated with refractory infection. Most common pathogens include
Staphylococcus aureus and
Aspergillus; hence, ST and itraconazole are administered for prevention. Prednisolone is additionally administered to CGD patients with severe infections, such as a liver abscess. Patients with primary antibody deficiency are treated with in travenous or subcutaneous IgRT. Serum IgG trough levels should be maintained at >700 mg/dL. Treatment for the prevention of infections for IEI depend on disease specificities and patient conditions.
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