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  2. Inherited autoinflammatory disease

Inherited autoinflammatory disease

Definition:

Inherited autoinflammatory diseases encompass a distinct and growing clinical entity of multisystem inflammatory diseases with known genetic defects in the innate immune system. NLRC4 inflammasomopathy [DS:H01748], Aicardi-Goutieres syndrome [DS:H00290], ADA2 deficiency [DS:H01382], and haploinsufficiency A20/TNFAIP3 [DS:H02592] are included.

Biomedical Dictionary

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