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Article

Excess Serum Interleukin-18 Distinguishes Patients With Pathogenic Mutations in PSTPIP1.

Arthritis & rheumatology (Hoboken, N.J.) - 1 Feb 2022

Stone Deborah L, Ombrello Amanda, Arostegui Juan I, Schneider Corinne, Dang Vinh, de Jesus Adriana, Girard-Guyonvarc'h Charlotte, Gabay Cem, Lee Wonyong, Chae Jae Jin, Aksentijevich Ivona, Goldbach-Mansky Raphaela T, Kastner Daniel L, Canna Scott W

Abstract excerpt

OBJECTIVE: Dominantly inherited PSTPIP1 mutations cause a spectrum of autoinflammatory manifestations epitomized by PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.). The connections between PSTPIP1 and PAPA syndrome are poorly understood, although evidence suggests involvement of pyrin inflammasome activation. Interleukin-18 (IL-18) is an inflammasome-activated cytokine...

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Identifiers

PMID: 34492165
DOI: 10.1002/art.41976
PMCID: PMC8855702

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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