Osial T A, Whiteside T L, Buckingham R B, Singh G, Barnes E L, Pierce J M, Rodnan G P
Arthritis Rheum. 1983 Apr;26(4):500-8. doi: 10.1002/art.1780260408.
Fifty-eight patients with progressive systemic sclerosis (PSS) were evaluated clinically and by biopsy of the minor salivary glands of the lips for the presence of Sjögren's syndrome. Clinical findings included dry eyes in 38%, dry mouth in 32%, parotid enlargement in 4%, and an abnormal Schirmer's test in 34%. Histologic changes in lip biopsies included lymphocytic infiltrates characteristic of Sjögren's syndrome in 17 individuals (29%). In 19 (33%) there was periglandular and intraglandular fibrosis (PSS-fibrosis) without significant inflammation, an alteration characteristic of PSS per se. In the remaining 22 patients (38%) with PSS, no abnormality was found. Of those with PSS and Sjögren's syndrome, 53% had serum antibodies to SS-A and/or SS-B, while only 1 patient with a normal biopsy had either of these antibodies. Anti-SS-A and anti-SS-B were not detected in patients with glandular fibrosis alone. The mortality rate of the PSS-fibrosis group was higher due to a variety of severe internal manifestations related to PSS. Antibodies to SS-A and SS-B are useful serologic markers of the presence of Sjögren's syndrome in patients with PSS.
对58例进行性系统性硬化症（PSS）患者进行了临床评估，并通过唇部小唾液腺活检来检查是否存在干燥综合征。临床发现包括38%的患者有干眼，32%有口干，4%有腮腺肿大，34%的患者施墨试验异常。唇部活检的组织学变化包括17例（29%）出现干燥综合征特征性的淋巴细胞浸润。19例（33%）出现腺周和腺内纤维化（PSS纤维化），无明显炎症，这是PSS本身的特征性改变。其余22例（38%）PSS患者未发现异常。在患有PSS和干燥综合征的患者中，53%有抗SS - A和/或抗SS - B血清抗体，而活检正常的患者中只有1例有这些抗体。仅患有腺纤维化的患者未检测到抗SS - A和抗SS - B。由于与PSS相关的各种严重内部表现，PSS纤维化组的死亡率较高。抗SS - A和抗SS - B抗体是PSS患者存在干燥综合征的有用血清学标志物。
