Sjögren's syndrome (SS) in patients with human T cell leukemia virus I associated myelopathy: paradoxical features of the major salivary glands compared to classical SS.

OBJECTIVE

To characterize imaging features of the major salivary glands in patients with human T cell leukemia virus I (HTLV-I) associated myelopathy (HAM) associated with Sjögren's syndrome (SS), and to compare these features with those in HAM negative patients with SS.

METHODS

The study population included 31 HAM patients (12 had associated SS), 15 HTLV-I seropositive/HAM negative patients with SS, and 41 HTLV-I seronegative patients with SS. Twenty HAM negative patients with sicca syndrome only were also studied. Diagnostic imaging (sialography, magnetic resonance 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 SS 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 SS. The labial glands from the HAM patients with SS exhibited significantly lower magnitudes of mononuclear cell aggregation compared with those in the HAM negative patients with SS. In contrast, all HAM negative patients with SS 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 SS in patients with HAM may occur in part via a mechanism distinctive from classical SS in HAM negative patients.