Background/Purpose: Sjögren syndrome is a complex autoimmune disease that affects lacrimal and salivary glands with the potential to cause damage to other organs. Diagnosis of childhood Sjögren syndrome (cSS) is currently based on expert opinion due to the lack of child-specific diagnostic criteria. Children do not typically present with 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 goal of this study was to compare clinical and serological profiles of cSS patients presenting with or without parotitis as a first step towards defining cSS diagnostic criteria.

Methods: We reviewed cSS 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 as well as extraglandular clinical features. For analyses of laboratory, serologic, imaging, pathology, and functional data, cases were excluded if specific data were not explicitly reported.

Results: We reviewed 26 cases of cSS diagnosed and followed at The Children’s Hospital of Philadelphia and 196 cases in the literature that contained information on individual children. Parotitis status was clearly indicated for 162 cases which were considered further. Parotitis was a main feature in presentation in 131 (81%) cases. Comparing the cases with parotitis (n=131) to those without (n=31), there were no differences in sex (78% and 87% female, respectively, P=0.448) or positive serologies: 87% and 90% ANA+, respectively (P=0.768), 72% and 61% RF+ (P=0.405), 86% and 90% SSA+ (P=0.761), 69% and 64% SSB+ (P=0.649). 53% of children had sicca symptoms regardless of parotitis status (P=1). Among extraglandular features, children without parotitis developed more joint symptoms (58% vs. 33%, P=0.012), CNS symptoms (32% vs. 7%, P=0.0003), Raynaud Phenomenon (16% vs. 5%, P=0.048), and renal tubular acidosis (16% vs. 4%, P=0.032). Non-significant extraglandular features included fever, rash, lymphadenopathy, fatigue, other renal manifestations, myositis, vaginal dryness, serositis, and transaminitis.

Conclusion: While the majority of childhood Sjögren syndrome (cSS) patients have parotitis, a significant percentage do not. Those who do not differ in their clinical features, with a significantly higher incidence of joint symptoms, RP, renal tubular acidosis, neuromyelitis optica, and all CNS manifestations. A large majority of all cSS patients have positive ANA and/or anti-SSA/B, and the serologic profiles do not significantly differ based on parotitis status. 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 cSS-specific diagnostic criteria are needed for future prospective studies to better characterize the prognosis and optimal therapies for this potentially devastating disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-clinical-and-serological-features-of-childhood-sjogren-syndrome-based-on-the-presence-or-absence-of-parotitis/