title: Sjögren syndrome in a child: favorable response of the arthritis to TNFalpha blockade.
abstract: Tumor necrosis factor alpha (TNFalpha) blockade has recently been found to be ineffective in treating glandular and extraglandular manifestations of adult Sjögren syndrome (SS), including arthralgia and arthritis. We report a girl who developed purpura, polyarthritis, uveitis, and severe dental caries in the first year of life and optic neuritis by age three. SS was diagnosed at 11 years of age, when severe hypokalemic renal tubular acidosis developed during infliximab treatment for arthritis. In contrast to her other disease manifestations, the arthritis responded remarkably well to TNFalpha blockade, suggesting that TNFalpha blockers may have a role in the treatment of arthritis with pediatric SS.
authors.affiliation: Division of Rheumatology, Department of Pediatrics, The Children's Hospital of Philadelphia, 3405 Civic Center Boulevard, Philadelphia, PA 19104, USA. pessler@email.chop.edu
authors.forename: F
authors.initials: F
authors.lastname: Pessler
authors.forename: B
authors.initials: B
authors.lastname: Monash
authors.forename: P
authors.initials: P
authors.lastname: Rettig
authors.forename: B
authors.initials: B
authors.lastname: Forbes
authors.forename: P A
authors.initials: PA
authors.lastname: Kreiger
authors.forename: R Q
authors.initials: RQ
authors.lastname: Cron
mesh_terms: D000141:Acidosis, Renal Tubular / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D001706:Biopsy; D002648:Child; D004359:Drug Therapy, Combination; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D011241:Prednisone / Q000627:therapeutic use; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D012470:Salivary Glands, Minor / Q000473:pathology; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept; D011241:Prednisone