Division of Pediatric Rheumatology, Department of Pediatrics, Duke University School of Medicine, MD 2301 Erwin Rd., Box #3212, Durham, NC, 27705, USA.
Duke Clinical Research Institute, Durham, NC, USA.
Pediatr Rheumatol Online J. 2022 Sep 5;20(1):79. doi: 10.1186/s12969-022-00740-4.
Sjögren disease in children and adolescents (pedSD) presents differently than adult disease. Diagnosis and classification are controversial, optimal treatment is unknown and outcomes are poorly understood. Here, we describe the current perspectives of pediatric rheumatologists on diagnosis, treatment, and outcomes of pedSD.
A voluntary, 17-question survey was distributed to providers in the Childhood Arthritis and Rheumatology Research Alliance and/or the American College of Rheumatology Childhood Sjögren's Study Group at the 2020 Convergence Virtual Conference. Findings are reported using descriptive statistics and chi-square testing.
Of 465 eligible providers, 157 (34%) responded with 135 (29%) completing the survey. The majority (85%) saw five or fewer patients with pedSD in the past year. Parotitis, dry eye and/or dry mouth, and constitutional symptoms were among the most specific and common clinical features. Most providers (77%) used clinical judgment guided by adult criteria for diagnosis. The vast majority (86-99%) of survey participants indicated routine use of serologic testing, while salivary gland ultrasound, minor salivary gland biopsy and other diagnostic tests were less often used. The most commonly prescribed systemic immunomodulators were hydroxychloroquine, corticosteroids, methotrexate, rituximab, and mycophenolate. Seven providers reported malignancy in a patient with pedSD, including one death.
Pediatric rheumatologists diagnose and treat pedSD; however, most only see a few patients per year and rely on clinical judgment and laboratory testing for diagnosis. Treatment frequently includes systemic immunomodulators and malignancies are reported. More studies are needed to better understand natural history, risk factors, and the impact of interventions on outcomes.
儿童和青少年干燥综合征（pedSD）的表现与成人疾病不同。诊断和分类存在争议，最佳治疗方法未知，预后也知之甚少。在此，我们描述了儿科风湿病医生对 pedSD 的诊断、治疗和预后的当前观点。
在 2020 年融合虚拟会议上，向儿童关节炎和风湿病研究联盟和/或美国风湿病学会儿童干燥综合征研究组的提供者自愿分发了一份包含 17 个问题的调查问卷。使用描述性统计和卡方检验报告结果。
在 465 名符合条件的提供者中，有 157 名（34%）做出了回应，其中 135 名（29%）完成了调查。大多数（85%）在过去一年中仅诊治过五例以下的 pedSD 患者。腮腺炎、干眼症和/或口干以及全身症状是最具特异性和最常见的临床特征。大多数（77%）提供者根据成人标准进行临床判断以诊断。绝大多数（86-99%）的调查参与者表示常规进行血清学检测，而唾液腺超声、唾液腺活检和其他诊断性检查则较少使用。最常开的系统性免疫调节剂为羟氯喹、皮质类固醇、甲氨蝶呤、利妥昔单抗和霉酚酸酯。有 7 名提供者报告了 pedSD 患者的恶性肿瘤，包括 1 例死亡。
儿科风湿病医生诊断和治疗 pedSD；然而，大多数医生每年仅诊治几例患者，并且依靠临床判断和实验室检测进行诊断。治疗常包括系统性免疫调节剂，并且报告了恶性肿瘤。需要进一步研究以更好地了解自然病史、风险因素以及干预对预后的影响。
