Besana C, Salmaggi C, Pellegrino C, Pierro L, Vergani S, Faravelli A, Rugarli C
Department of Internal Medicine, Università di Milano.
Eur J Pediatr. 1991 Jul;150(9):652-5. doi: 10.1007/BF02072627.
We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mono-nuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjögren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses. Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.
我们报告了一例10岁同卵双胞胎姐妹患慢性双侧泪腺炎的罕见病例。两个女孩均表现为双侧泪腺肿大，并出现中度干眼症和角膜炎。泪腺和小唾液腺活检均显示单核细胞非肉芽肿性炎性浸润。因此可以排除所有肉芽肿性疾病和肿瘤，仅干燥综合征和极少数其他形式的慢性泪腺炎仍有可能是诊断结果。对两名患者及其父母进行了自身抗体检测。在其中一人中发现了极低滴度的平滑肌抗体，两人中有抗核抗体，在该家庭的所有四名成员中均发现了抗双链DNA抗体。尽管其中一名姐妹的抗核抗体和抗双链DNA抗体滴度有所升高，但两名患者均未出现任何系统性结缔组织病的体征或症状。在6年的随访期间，两名患者均表现为睑板腺持续肿大，但除中度干眼症和偶尔的轻度角膜炎外，无其他症状。
