Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade

ADULT-ONSET PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS

(2010) Marie Cavuoto et al.   ANNALS OF ALLERGY ASTHMA & IMMUNOLOGY

Horror Autoinflammaticus: The Molecular Pathophysiology of Autoinflammatory Disease

(2009) Seth L. Masters et al.   Annual Review of Immunology

Immunological and Inflammatory Functions of the Interleukin-1 Family

(2009) Charles A. Dinarello   Annual Review of Immunology

Outcome of Tonsillectomy in Selected Patients With PFAPA Syndrome

(2009) Lorenzo Pignataro et al.   ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY

Subtype-specific peripheral blood gene expression profiles in recent-onset juvenile idiopathic arthritis

(2009) Michael G. Barnes et al.   ARTHRITIS AND RHEUMATISM

Novel markers of inflammation identified in tumor necrosis factor receptor-associated periodic syndrome (TRAPS) by transcriptomic analysis of effects of TRAPS-associated tumor necrosis factor receptor type I mutations in an endothelial cell line

(2009) Susana L. Rebelo et al.   ARTHRITIS AND RHEUMATISM

Effectiveness of Adenotonsillectomy in PFAPA Syndrome: A Randomized Study

(2009) Werner Garavello et al.   JOURNAL OF PEDIATRICS

AIM2 activates the inflammasome and cell death in response to cytoplasmic DNA

(2009) Teresa Fernandes-Alnemri et al.   NATURE

AIM2 recognizes cytosolic dsDNA and forms a caspase-1-activating inflammasome with ASC

(2009) Veit Hornung et al.   NATURE

An orthogonal proteomic-genomic screen identifies AIM2 as a cytoplasmic DNA sensor for the inflammasome

(2009) Tilmann Bürckstümmer et al.   NATURE IMMUNOLOGY

Differentiating PFAPA Syndrome From Monogenic Periodic Fevers

(2009) M. Gattorno et al.   PEDIATRICS

Effect of Adenotonsillectomy in PFAPA Syndrome

(2008) Greg Licameli et al.   ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome is not always successful

(2008) Takeshi Ogose   JOURNAL OF PEDIATRICS

Periodic Fever accompanied by Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome (PFAPA syndrome) in adults

(2008) S Padeh et al.   Pediatric Rheumatology