Variable expression and treatment of PAPA syndrome

Annals of the Rheumatic Diseases

Volumn 70, Issue 6, 2011, Pages 1168-1170

  Schellevis, Meike A ^a   Stoffels, Monique ^a   Hoppenreijs, Esther P A H ^a   Bodar, Evelien ^a   Simon, Anna ^a   Van Der Meer, Jos W M ^a  

^a RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLSALICYLIC ACID; ANTIBIOTIC AGENT; ANTIDIABETIC AGENT; C REACTIVE PROTEIN; CORTICOSTEROID; IBUPROFEN; PREDNISONE; RECOMBINANT INTERLEUKIN 1 RECEPTOR BLOCKING AGENT;

ABDOMINAL PAIN; ACNE; ADOLESCENT; ADULT; ANTIPHOSPHOLIPID SYNDROME; ARTHRITIS; CASE REPORT; CHILD; CLINICAL FEATURE; FEMALE; FEVER; HIDRADENITIS; HUMAN; INJECTION SITE PAIN; LETTER; PAPA SYNDROME; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PUSTULOSIS; TREATMENT OUTCOME;

ACNE VULGARIS; ADULT; ANTIRHEUMATIC AGENTS; ARTHRITIS, INFECTIOUS; CHILD; CYTOKINES; FEMALE; HUMANS; INTERLEUKIN 1 RECEPTOR ANTAGONIST PROTEIN; MIDDLE AGED; PREGNANCY; PREGNANCY COMPLICATIONS; PYODERMA GANGRENOSUM;

EID: 79955825388     PISSN: 00034967     EISSN: 14682060     Source Type: Journal    
DOI: 10.1136/ard.2009.126185     Document Type: Letter

References (8)

1. Lindor NM, Arsenault TM, Solomon H, et al. A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome. Mayo Clin Proc 1997;72:611-15. (Pubitemid 27330245) (Pubitemid 27330245)
2. Cortis E, De Benedetti F, Insalaco A, et al. Abnormal production of tumor necrosis factor (TNF) -alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected]. J Pediatr 2004;145:851-5. (Pubitemid 39586631) (Pubitemid 39586631)
3. Dierselhuis MP, Frenkel J, Wulffraat NM, et al. Anakinra for flares of pyogenic arthritis in PAPA syndrome. Rheumatology (Oxford) 2005;44:406-8. (Pubitemid 40361307) (Pubitemid 40361307)
4. Stichweh DS, Punaro M, Pascual V. Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome. Pediatr Dermatol 2005;22:262-5. (Pubitemid 40741268) (Pubitemid 40741268)
5. Yeon HB, Lindor NM, Seidman JG, et al. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome maps to chromosome 15q. Am J Hum Genet 2000;66:1443-8. (Pubitemid 30468802) (Pubitemid 30468802)
6. Wise CA, Gillum JD, Seidman CE, et al. Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder. Hum Mol Genet 2002;11:961-9. (Pubitemid 34449783) (Pubitemid 34449783)
7. Simon A, van der Meer JW. Pathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes. Am J Physiol Regul Integr Comp Physiol 2007;292:R86-98. (Pubitemid 46089817) (Pubitemid 46089817)
8. Hirschfeld M, Weis JJ, Toshchakov V, et al. Signaling by toll-like receptor 2 and 4 agonists results in differential gene expression in murine macrophages. Infect Immun 2001;69:1477-82. (Pubitemid 32187597) (Pubitemid 32187597)