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

Proceedings of the National Academy of Sciences of the United States of America

Volumn 108, Issue 17, 2011, Pages 7148-7153

  Stojanov, Silvia ^a,e   Lapidus, Sivia ^a   Chitkara, Puja ^a   Feder, Henry ^f   Salazar, Juan C ^f   Fleisher, Thomas A ^b   Brown, Margaret R ^b   Edwards, Kathryn M ^g   Ward, Michael M ^a   Colbert, Robert A ^a   Sun, Hong Wei ^a   Wood, Geryl M ^a,c   Barham, Beverly K ^a,c   Jones, Anne ^a,c   Aksentijevich, Ivona ^a,c   Goldbach Mansky, Raphaela ^a   Athreya, Balu ^h   Barron, Karyl S ^d   Kastner, Daniel L ^a,c  

^a NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES   (United States)

^b NATIONAL INSTITUTES OF HEALTH   (United States)

^c NATIONAL HUMAN GENOME RESEARCH INSTITUTE   (United States)

^d NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES   (United States)

^e UNIVERSITY OF MUNICH   (Germany)

^f UNIVERSITY OF CONNECTICUT HEALTH CENTER   (United States)

^g VANDERBILT UNIVERSITY SCHOOL OF MEDICINE   (United States)

^h NEMOURS ALFRED I DUPONT HOSPITAL FOR CHILDREN   (United States)

Author keywords

Anakinra; Autoinflammatory disease; Inflammasome; Therapy

Indexed keywords

BIOLOGICAL MARKER; C REACTIVE PROTEIN; CD3 ANTIGEN; CD8 ANTIGEN; CIMETIDINE; COMPLEMENT COMPONENT C1Q; COMPLEMENT COMPONENT C2; CXCL9 CHEMOKINE; GAMMA INTERFERON INDUCIBLE PROTEIN 10; GRANULOCYTE COLONY STIMULATING FACTOR; IBUPROFEN; INFLAMMASOME; INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; INTERLEUKIN 18; INTERLEUKIN 1BETA; INTERLEUKIN 1BETA CONVERTING ENZYME; INTERLEUKIN 6; MACROPHAGE INFLAMMATORY PROTEIN 1BETA; NEUROSERPIN; PARACETAMOL; PROTEIN AIM2; RECOMBINANT INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; UNCLASSIFIED DRUG;

ARTICLE; CD4+ CD25+ T LYMPHOCYTE; CD4+ T LYMPHOCYTE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; CYTOKINE PRODUCTION; DISEASE EXACERBATION; FEMALE; GENE EXPRESSION PROFILING; GENE EXPRESSION REGULATION; HEREDITARY PERIODIC FEVER; HUMAN; INNATE IMMUNITY; LYMPHOCYTE COUNT; LYMPHOCYTOPENIA; MALE; PATHOGENESIS; PFAPA SYNDROME; PRIORITY JOURNAL; RECURRENT FEVER; SYSTEMS BIOLOGY; T LYMPHOCYTE ACTIVATION; TH1 CELL; TREATMENT RESPONSE;

ADOLESCENT; CHILD; CHILD, PRESCHOOL; CYTOKINES; FEMALE; FEVER; GENE EXPRESSION PROFILING; GENE EXPRESSION REGULATION; HUMANS; IMMUNITY, INNATE; INTERLEUKIN-1; LYMPHADENITIS; LYMPHOCYTE ACTIVATION; MALE; PHARYNGITIS; STOMATITIS, APHTHOUS; TH1 CELLS;

EID: 79955147587     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1103681108     Document Type: Article

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