Clin Rheumatol (2006) 25: 746–748 DOI 10.1007/s10067-005-0042-z CASE REPORT F. Pessler . B. Monash . P. Rettig . B. Forbes . P. A. Kreiger . R. Q. Cron Sjögren syndrome in a child: favorable response of the arthritis to TNFα blockade Received: 8 March 2005 / Accepted: 14 April 2005 / Published online: 4 January 2006 # Clinical Rheumatology 2006 Abstract Tumor necrosis factor alpha (TNFα) blockade has recently been found to be ineffective in treating glandular and extraglandular manifestations of adult Sjögren syn- drome (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 re- markably well to TNFα blockade, suggesting that TNFα blockers may have a role in the treatment of arthritis with pediatric SS. Keywords Arthritis . Optic neuritis . Pediatric Sjögren syndrome . Renal tubular acidosis . Sjögren syndrome . Tumor necrosis factor alpha . Uveitis Abbreviations JRA: Juvenile rheumatoid arthritis . RA: Rheumatoid arthritis . RTA: Renal tubular acidosis . SS: Sjögren syndrome . TNFα: Tumor necrosis factor alpha Introduction Sjögren syndrome (SS) is reportedly milder in children than in adults insofar as the most frequent primary symptom is benign parotid swelling, whereas the sicca syndrome occurs later and less frequently [1]. Nonetheless, some of the potentially severe, systemic complications of adult SS have also been described in pediatric patients [2, 3], including renal tubular acidosis (RTA) [4] and optic neuritis [5]. The treatment of SS is primarily symptom oriented, and no single systemic disease-modifying agent exists. Nonste- roidal antiinflammatory drugs (NSAIDs) and glucocorti- coids form the mainstay of systemic treatment. Tumor necrosis factor alpha (TNFα) inhibitors are ef- fective for the treatment of rheumatoid arthritis (RA) [6], polyarticular juvenile rheumatoid arthritis (JRA) (reviewed in Reiff [7]), and other inflammatory conditions with high TNFα activity. TNFα has been implicated in glandular destruction from SS [8]. Surprisingly, recent trials showed no benefit of TNFα blockade in glandular and extra- glandular manifestations of primary adult SS, except a mild reduction of fatigue [9–11]. We describe a child with SS, complicated by xerostomia, uveitis, optic neuritis, RTA, and chronic polyarthritis. Her arthritis improved remarkably with TNFα blockade. In contrast, there was no improvement of her other SS-asso- ciated disease manifestations. Indeed, severe RTA, a known extraglandular manifestation of SS, developed during infli- ximab treatment. This case underscores that pediatric SS can take a severe course and reinforces the importance of its early recognition and treatment. Moreover, it suggests a F. Pessler (*) . P. Rettig . R. Q. Cron Division of Rheumatology, Department of Pediatrics, The Children’s Hospital of Philadelphia, 3405 Civic Center Boulevard, Philadelphia, PA 19104, USA e-mail: pessler@email.chop.edu Tel.: +1-215-5907180 Fax: +1-215-5904750 B. Monash . B. Forbes . R. Q. Cron School of Medicine, University of Pennsylvania, 295 John Morgan Building, 3620 Hamilton Walk, Philadelphia, PA 1910, USA B. Forbes Division of Ophthathalmology, Department of Pediatrics, The Children’s Hospital of Philadelphia, 3405 Civic Center Boulevard, Philadelphia, PA 19104, USA P. A. Kreiger Department of Pathology, The Children’s Hospital of Philadelphia, 3405 Civic Center Boulevard, Philadelphia, PA 19104, USA