CD4-LYMPHOCYTOPENIA WITHOUT HIV-INFECTION - INCREASED PREVALENCE AMONG PATIENTS WITH PRIMARY SJOGRENS-SYNDROME( T)

Objective. Primary Sjogren's syndrome (1 degrees SS) is an autoimmune disease, usually accompanied by manifest immune hyperactivity. In some cases the disease converts to malignant neoplasia. On the other hand, there are clinical similarities to HN infection. Since the I-are phen omenon of persistent depletion of CD4+ T-lymphocytes in peripheral blo od without HIV infection was recently defined as idiopathic CD4+ T-lym phocytopenia (ICL), we have used the ICL criteria to investigate the p revalence of this phenomenon among 1 degrees SS patients. Methods. Dur ing the period 1988-94, 115 caucasian patients (10 males), mean age 57 .8 (range 19 - 82) years, with 1 degrees SS were prospectively studied Lymphocyte subsets were investigated by means of monoclonal antibodie s and flow cytometry. For the detection of HIV and HTLV antibodies, we used an enzyme immunoassay (for HIV-1 and HIV-2), Western blot techni ques (HN-I, HIV-2, HTLV-I and HTLV-II), and the polymerase chain react ion procedure (HIV-1, HTLV-I and HTLV-II). HN antigens were tested for with the HN-I p-24 Ag test. Results. Six patients with 1 degrees SS f ulfilled the criteria for ICL. While the clinical condition of 5 of th ose six patients remained stable, one patient developed malignant lymp homa three years after her disease was classified as a case of ICL. Th e prevalence of ICL among our 115 patients with 1 degrees SS was 5.2%, which is significantly higher than the rates reported for arty other patient or population group. We have estimated the relative risk of IC L in 1 degrees SS patients to vary from 3.4 to 6,000 (P values of 0.00 01 - 0.025). Conclusion. We suggest that subjects with ICL should be c arefully examined for 1 degrees SS and, if its presence is confirmed, that they should be followed with regard to the possible complications of this disease, including the development of malignant lymphoma.