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Article

Diagnosis and management of ADA2 deficient polyarteritis nodosa.

International journal of rheumatic diseases - 1 Jan 2019

Human Andrea, Pagnoux Christian

Abstract excerpt

Deficiency of ADA2 (DADA2) is a recently described systemic inflammatory vasculopathy caused by mutations in the CERC1 gene that often, but not always, clinically resembles polyarteritis nodosa (PAN). The condition was originally characterized by livedoid rash, systemic inflammation, variable hypogammaglobulinemia, and early-onset stroke. The phenotypic spectrum has expanded to include patients with...

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Identifiers

PMID: 29624883
DOI: 10.1111/1756-185X.13283

Genes

ADA2

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Diagnosis and management of ADA2 deficient polyarteritis nodosa.PMID 29624883Read ↗

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