Sjogren's Syndrome in patients with HTLV-I-associated myelopathy(HAM) :

目的：描述与干燥综合征相关的HTLV-Ⅰ相关脊髓病（HAM）患者的大唾液腺影像学特征，并与HAM阴性的干燥综合征患者进行比较。主题和方法。该研究包括31例HAM患者（12例与Sjogren综合征相关），15例HTLV-I血清阳性/HAM阴性的Sjogren综合征患者，41例HTLV-I血清阴性的Sjogren综合征患者。另外还研究了20例仅患有干燥综合征的HAM阴性患者。唾液腺的诊断性成像（涎腺造影、MR成像和超声检查）。对患者进行唇腺活检、Schirmer试验、Saxon试验和血清学检查。结果12例HAM患者中有11例（92%）的腮腺和下颌下腺完全缺乏该疾病的异常影像学特征，而唾液流速降低的水平与HAM阴性的Sjogren综合征患者相似。与HAM阴性的干燥综合征患者相比，HAM干燥综合征患者的唇腺表现出显著较低的单核细胞聚集程度。与此相反，所有HAM阴性的干燥综合征患者均表现出该疾病特有的异常影像学特征，且唾液分泌功能障碍的严重程度与影像学表现密切相关。这些结果表明，舍格伦综合征在HAM患者可能至少部分发生通过一个独特的机制，从经典舍格伦综合征在HAM阴性患者。

Objective.To characterize imaging features of the major salivary glands in patients with HTLV-I-associated myelopathy(HAM) who were associated with Sjogren's syndrome, and to compare these features with those in HAM-negative patients with Sjogren's syndrome. Subjects and Methods. The study propulation included 31 HAM patients(12 were associated with Sjogren's syndrome), 15 HTLV-I-seropositive/HAM-negative patients with Sjogren's syndrome, and 41 HTLV-I-seronegative patients with Sjogren's syndrome. Separate 20 HAM-negative patients with sicca syndrome only were also studied. Diagnostic imaging(sialography, MR imaging, and sonography) of the salivary glands. labial gland biopsy, Schirmer test, Saxon test and serological tests were performed on these patients. Results. The parotid and submandibular glands in 11(92%) of the 12 HAM patients with Sjogren's syndrome completely lacked the abnormal imaging features characteristic of the disease, while they displayed decreased salivary flow rates at levels similar to those in the HAM-negative patients with Sjogren's Syndrome. The labial glands from the HAM patients with Sjogren's syndrome exhibited significantly lower magnitudes of mononuclear cell aggregation compared with those in the HAM-negative patients with Sjogren's syndrome. In contrast, all HAM-negative patients with Sjogren's syndrome showed abnormal imaging features characteristic of the disease, and the severity in salivary dysfunction correlated well with the imaging findings.Conclusion. These results suggest that Sjogren's syndrome in HAM patients may at least in part occur via a distinctive mechanism from classical Sjogren's syndrome in HAM-negative patients.