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Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies

ORPHA:306431· ICD-10 D84.8

Definition

A rare acquired immunodeficiency disorder characterized by the appearance of susceptibility to disseminated opportunistic infections (in particular, disseminated nontuberculous mycobacterial infection, salmonellosis, penicillosis, and varicella zoster virus infection) in previously healthy (HIV-negative) adults, associated with the presence of acquired autoantibodies to interferon gamma. Typical clinical manifestation includes lymphadenopathy (cervical or generalized), fever, weight loss and/or reactive skin lesions.

Prevalence
Unknown
Inheritance
Not applicable
Age of onset
Adult