A73: A Comparison of Serologic Profiles of Children With Sjögren Syndrome Based on the Presence or Absence of Parotitis. Issue Volume 66:Issue(2014)supplement 3 (March 2014)

• Record Type: Journal Article
• Authors: Mehta, Jay; Yokogawa, Naoto; Lieberman, Scott
• Is Part Of: Arthritis & rheumatology. Volume 66:Issue(2014)supplement 3
• Page Start: S105
• Page End: S105
• Publication Date: 2014-03
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.38489
• Languages: English
• ISSNs: 2326-5191

Abstract

Abstract : Background/Purpose: Sjögren syndrome is a complex autoimmune disease that affects lacrimal and salivary glands along with the potential to cause damage to other organs. Diagnosis of childhood Sjögren syndrome is currently based on expert opinion due to the lack of child‐specific diagnostic criteria. Children do not typically present with the sicca symptoms characteristic of adult Sjögren syndrome. While they most commonly present with recurrent parotitis, some children present with other organ involvement in the absence of parotitis. The latter may be severe requiring more extensive immunomodulatory therapy. The goal of this study was to compare serologic profiles of cohorts of children with Sjögren syndrome presenting with or without parotitis to determine if separate serology‐based diagnostic criteria are needed for these two groups. Methods: We retrospectively reviewed childhood Sjögren syndrome cases from a single center as well as those in the published English literature with individual patient data. We collected available data on sicca symptoms, serologies (ANA, anti‐SSA/B, RF), lacrimal and salivary gland function, imaging, and histopathology. For analyses of individual components, cases were excluded if specific data was not explicitly reported, so denominators may vary. Results: We identified 26 cases of childhood Sjögren syndrome diagnosed and followed at The Children's Hospital of Philadelphia and 67 cases in the literature that contained information on individual children. Parotitis status was reported for 82 cases and was a main feature of presentation in 59 (72%) cases. 28 of 51 (55%) children with parotitis and 13 of 22 (59%) children without parotitis presented with sicca symptoms. 53 of 58 (91%) children with parotitis and 21 of 23 (91%) children without parotitis were ANA positive. 49 of 58 (84%) children with parotitis and 20 of 23 (87%) children without parotitis were anti‐SSA/B positive. 42 of 51 (82%) children with parotitis and 7 of 13 (54%) children without parotitis were RF positive. 56 of 58 (97%) children with parotitis and 22 of 23 (96%) children without parotitis were positive for either ANA or anti‐SSA/B. Conclusion: A large majority (96%) of children with Sjögren syndrome have positive ANA and/or anti‐SSA/B, and the serologic profiles do not significantly differ based on the presence or absence of parotitis. Thus, testing for ANA and anti‐SSA/B is warranted in the diagnostic workup of any child suspected of having Sjögren syndrome, especially in patients who do not present with parotitis. Given the selection bias inherent in this approach, development of childhood Sjögren syndrome‐specific diagnostic criteria are needed for future prospective studies to better characterize the prognosis and optimal therapies for this potentially devastating disease.