HIGH PREVALENCE OF SJOGRENS-SYNDROME IN PATIENTS WITH HTLV-I-ASSOCIATED MYELOPATHY

Objective - A high seroprevalence of HTLV-I in female Sjogren's syndro me (SS) patients has been reported in Nagasaki, Japan, an area that is heavily endemic for HTLV-I infection. Salivary IgA class antibodies t o HTLV-I were common among HTLV-I seropositive patients with SS. This study was undertaken to elucidate the pathogenesis of SS caused by HTL V-I infection. Methods - The clinical features and histological findin gs of SS and the prevalence of serum autoantibodies in 10 patients wit h HTLV-I associated myelopathy (HAM) who were consecutively admitted i nto Nagasaki University School of Medicine, were compared with those o f 20 HTLV-I seropositive and 20 HTLV-I seronegative patients with SS. Results - Ocular and oral manifestations of SS were commonly detected in HAM patients. These patients also had extraglandular manifestations including recurrent uveitis, arthropathy, interstitial pneumonitis, R aynaud's phenomenon, and inflammatory bowel disease. All patients with HAM histologically showed a mononuclear cell infiltration in the labi al salivary grands. Six of 10 patients had a mononuclear cell infiltra tion with a focus score of 1 or greater. According to the preliminary criteria for SS proposed by the European Community, definitive SS was diagnosed in six patients and probable SS in two patients. Serum gamma globulin and IgG values were increased in HAM patients. Patients with HAM had lower prevalence of rheumatoid factor, antinuclear antibody, and anti-SS-A (Re) antibody than those of HTLV-I seropositive and HTLV -I seronegative SS patients. However, there was no significant differe nce in the prevalence of these antibodies among HAM patients with defi nitive SS, HTLV-I seropositive and HTLV-I seronegative SS patients. Th e CD3(+)CD4(+) T cells preferentially infiltrated into the salivary gl ands in HAM patients as well as the salivary glands of patients with H TLV-I seropositive and seronegative patients. It seems probable that p eripheral blood mononuclear cells from HAM patients preferentially inf iltrated into the salivary glands, and that these cells produced the a utoantibodies as well as anti-HTLV-I antibody. Conclusion - The result s strongly support the idea that HTLV-I is involved in the pathogenesi s of the disease in a subset of patients with SS in endemic areas.