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Section 6. Immunologic Diagnosis of Primary Immune
Dysregulation Disorders and Autoinflammatory Disorders
Recommendation 6.1
➤ We recommend evaluation for IEI in patients with clinical
manifestations of immune dysregulation, such as immunodeficiency,
autoimmunity, lymphoproliferation, and autoinflammation. (S-H)
Recommendation 6.2
➤ We recommend the assessment of cellular and humoral immunologic
functions in patients with suspected primary immune dysregulation
disorders. (S-H)
Recommendation 6.3
➤ We recommend that patients with periodic fevers and chronic systemic
inflammation should be evaluated for IEI and secondary causes such as
infection, autoimmune disease, or malignancy. (S-H)
Recommendation 6.4
➤ We recommend that patients who exhibit lymphoproliferation and
autoimmunity be evaluated for primary and secondary immune
dysregulation syndromes. (S-H)
