Houghton Kristin M, Cabral David A, Petty Ross E, Tucker Lori B
Division of Rheumatology, British Columbia's Children's Hospital and University of British Columbia, Vancouver, Canada.
J Rheumatol. 2005 Aug;32(8):1603-6.
Primary Sjögren's syndrome (pSS) is uncommonly recognized in childhood, and familial cases are rare. Pulmonary involvement in pediatric pSS is infrequently reported. In adults, asymptomatic pulmonary involvement is increasingly recognized, manifest by pulmonary function test abnormalities and changes on high resolution computerized tomographic scan. We describe a case of pSS in a 14-year-old Vietnamese-Canadian girl who presented with pulmonary symptoms, radiologic changes, and biopsy confirmation of lymphocytic interstitial pneumonia. Her dizygotic twin sister has primary SS without extraglandular manifestations. To our knowledge this is the first report of pediatric pSS with lymphocytic interstitial pneumonia and multiple pulmonary nodules on chest radiograph. We review the literature on pulmonary involvement and familial cases of pSS in childhood.
原发性干燥综合征（pSS）在儿童时期很少被认识到，家族性病例也很罕见。小儿pSS的肺部受累情况鲜有报道。在成人中，无症状肺部受累越来越受到重视，表现为肺功能测试异常和高分辨率计算机断层扫描的改变。我们描述了一名14岁越南裔加拿大女孩的pSS病例，她出现了肺部症状、放射学改变以及淋巴细胞间质性肺炎的活检证实。她的异卵双胞胎姐妹患有原发性干燥综合征，但无腺外表现。据我们所知，这是第一例小儿pSS伴有淋巴细胞间质性肺炎且胸部X线片上有多个肺结节的报告。我们回顾了有关小儿pSS肺部受累及家族性病例的文献。
