Department of Medicine, Case Western Reserve University, MetroHealth Campus, Cleveland, OH, USA.
Clin Rheumatol. 2010 Jul;29(7):789-91. doi: 10.1007/s10067-009-1359-9. Epub 2010 Jan 19.
Primary Sjogren's syndrome (pSS) is a chronic autoimmune disorder of the exocrine glands. The diagnosis is largely based on keratoconjunctivitis sicca and xerostomia in the presence of anti-SS-A and/or SS-B antibodies. Anticentromere antibodies (ACA) have occasionally been reported in patients with pSS. We describe two patients with pSS associated with ACA, initially diagnosed as limited systemic sclerosis. Symptoms at the time of initial presentation were dry eyes and mouth, arthralgias, and Raynaud's phenomenon. Both patients developed small vessel cutaneous vasculitis, parotid enlargement, low C4 complement levels, positive rheumatoid factor, and lymphoma. These findings suggest that patients with pSS who have ACA may be a subgroup of patients at increased risk of extraglandular systemic manifestations and lymphoma.
原发性干燥综合征（pSS）是一种慢性外分泌腺自身免疫性疾病。其诊断主要基于存在抗 SS-A 和/或 SS-B 抗体时的干燥性角结膜炎和口干。偶尔有报道称抗着丝点抗体（ACA）存在于 pSS 患者中。我们描述了两例与 ACA 相关的 pSS 患者，最初被诊断为局限性系统性硬化症。初始表现时的症状为眼睛和嘴巴干燥、关节痛和雷诺现象。两名患者均出现小血管皮肤血管炎、腮腺肿大、C4 补体水平降低、类风湿因子阳性和淋巴瘤。这些发现表明，患有 ACA 的 pSS 患者可能是发生外分泌腺系统表现和淋巴瘤风险增加的亚组患者。
