Lower frequency of focal lip sialadenitis (focus score) in smoking patients. Can tobacco diminish the salivary gland involvement as judged by histological examination and anti-SSA/Ro and anti-SSB/La antibodies in Sjögren's syndrome?

OBJECTIVES: Prospectively collected computer database information was previously assessed on a cohort of 300 patients who fulfilled the Copenhagen classification criteria for primary Sjögren's syndrome. Analysis of the clinical data showed that patients who smoked had a decreased lower lip salivary gland focus score (p<0.05). The aim of this original report is to describe the tobacco habits in patients with primary Sjögren's syndrome or stomatitis sicca only and to determine if there is a correlation between smoking habits and focus score in lower lip biopsies as well as ciculating autoantibodies and IgG. METHODS: All living patients with primary Sjögren's syndrome or stomatitis sicca only, who were still in contact with the Sjögren's Syndrome Research Centre were asked to fill in a detailed questionnaire concerning present and past smoking habits, which was compared with smoking habits in a sex and age matched control group (n=3700) from the general population. In addition, the patients previous lower lip biopsies were blindly re-evaluated and divided by the presence of focus score (focus score = number of lymphocyte foci per 4 mm(2) glandular tissue) into those being normal (focus score 1). Furthermore the cohort was divided into three groups; 10-45, 46-60 and >/= 61 years of age. Finally the focus score was related to the smoking habits. Seroimmunological (ANA; anti-SSA/Ro antibodies; anti-SSB/La antibodies; IgM-RF and IgG) samples were analysed routinely. RESULTS: The questionnaire was answered by 98% (n=355) of the cohort and the percentage of current smokers, former smokers and historical non-smokers at the time of lower lip biopsy was not statistically different from that of the control group. Cigarette smoking at the time of lower lip biopsy is associated with lower risk of abnormal focus score (p<0.001; odds ratio 0.29, 95%CI 0.16 to 0.50). The odds ratio for having focal sialadenitis (focus score > 1) compared with having a non-focal sialadenitis or normal biopsy (focus score /= 61: odds ratio 0.36, 95%CI 0.10 to 1.43) although there was only statistical significance in the two younger age groups. Moreover, among current smokers at the time of the lower lip biopsy there was a decreasing odds ratio for an abnormal lip focus score with increasing number of cigarettes smoked per week (p trend 0.00). In the group of former smokers, which included patients that had stopped smoking up to 30 years ago, the results were in between those of the smokers and the historical non-smokers (odds ratio 0.57, 95%CI 0.34 to 0.97, compared with never smokers). Present or past smoking did not correlate with the function of the salivary glands as judged by unstimulated whole sialometry, stimulated whole sialometry or salivary gland scintigraphy. Among former smokers, the median time lapse between the first symptom of primary Sjögren's syndrome and the performance of the lower lip biopsy was approximately half as long as the median time lapse between smoking cessation and biopsy (8 versus 15 years). Hence, symptoms of Sjögren's syndrome are unlikely to have had a significant influence on smoking habits at the time of the biopsy. Among the seroimmunological results only anti-SSA/Ro and anti-SSB/La antibodies reached statistical significance in a manner similar to the way smoking influenced the focus score in lower lip biopsies. On the other hand the level of significance was consistently more pronounced for the influence of smoking on the focus score than for the influence on anti-SSA/Ro and anti-SSB/La autoantibodies. CONCLUSION: This is believed to be the first report showing that cigarette smoking is negatively associated with focal sialadenitis-focus score >1-in lower lip biopsy in patients with primary Sjögren's syndrome. Furthermore, tobacco seems to decrea

Telemedicine and distance learning--experience in Georgia and future prospects.

Telemedicine and distance learning have a high priority in Georgia owing to the uneven distribution of health-care resources. Outside the country's capital, Tbilisi, resources and manpower are scarce, so telemedicine may be the best option for health-care delivery in spite of the costs. However, an adequate infrastructure must be in place to make this possible. The National Information Learning Centre (NILC) provides access to the Internet and MEDLINE and is currently assessing the best software for low-bandwidth distance learning and videoconferencing.

Epidemiology of Sjögren's syndrome, especially its primary form.

During the last decades different sets of criteria have been used for defining primary Sjögren's syndrome. Using these criteria prevalence estimates have varied between 0.04%-4.8%. This hundred-fold distribution in estimated frequency is partly due to differences in age groups studied, classification criteria used and methods used for objective evaluation of lachrymal and salivary gland hypofunction. Another problem which makes comparison between studies difficult is that workshop recommendations for the various objective tests are very often not strictly followed. Population based studies tend to identify cases with less severe disease and higher prevalence rates. Furthermore, there seems to be little correlation between dryness symptoms reported by subjects and objective hypofunction of lachrymal and/or salivary glands. Possible explanations to these findings include selection bias towards more severe cases in clinical (university) studies and a low predictive value for some criteria in population studies.

CD4+ T-lymphocytopenia without HIV infection: increased prevalence among patients with primary Sjögren's syndrome.

OBJECTIVE: Primary Sjögren's syndrome (1 degree 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 HIV infection. Since the rare phenomenon of persistent depletion of CD4+ T-lymphocytes in peripheral blood without HIV infection was recently defined as idiopathic CD4+ T-lymphocytopenia (ICL), we have used the ICL criteria to investigate the prevalence of this phenomenon among 1 degree SS patients. METHODS: During the period 1988-94, 115 caucasian patients (10 males), mean age 57.8 (range 19-82) years, with 1 degree SS were prospectively studied. Lymphocyte subsets were investigated by means of monoclonal antibodies and flow cytometry. For the detection of HIV and HTLV antibodies, we used an enzyme immunoassay (for HIV-1 and HIV-2), Western blot techniques (HIV-1, HIV-2, HTLV-I and HTLV-II), and the polymerase chain reaction procedure (HIV-1, HTLV-I and HTLV-II). HIV antigens were tested for with the HIV-1 p-24 Ag test. RESULTS: Six patients with 1 degree SS fulfilled the criteria for ICL. While the clinical condition of 5 of those six patients remained stable, one patient developed malignant lymphoma three years after her disease was classified as a case of ICL. The prevalence of ICL among our 115 patients with 1 degree SS was 5.2%, which is significantly higher than the rates reported for any other patient or population group. We have estimated the relative risk of ICL in 1 degree SS patients to vary from 3.4 to 6,000 (P values of 0.0001-0.025). CONCLUSION: We suggest that subjects with ICL should be carefully examined for 1 degree 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.

[The clinical significance of disorders of humoral immunity in Sjögren's disease and syndrome]. / Klinicheskoe znachenie narushenii gumoral'nogo immuniteta pri bolezni i sindrome Shegrena.

Data on 100 patients: 50 with Sjögren's disease (SD) and 50 with Sjögren's syndrome (SS) accompanied by systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic scleroderma (SSD) were analysed. It was characteristic of SD to have hyperproteinemia, hypergammaglobulinemia, high levels of the rheumatoid factor (RF), circulating immune complexes (CIC), and cryoglobulins, anti-Ro and anti-La; SD + RA was characterized by RF, hypercomplementemia; SS + SLE was characterized by various antibodies to nucleic acids (nDNA, DNP (and anti-Sm, antinuclear factor (ANF) of homogenous and peripheral types of glow, hypercomplementemia. The main systemic manifestations of SD (arthritis, vasculitis) and a high activity of the process was associated with the presence of anti-Ro/La, and marked local manifestations of the dry syndrome--with antibodies to nDNA and dDNA. And vice versa in SS + SLE anti-nDNA antibodies correlated with anemia, serositis, nephritis, vasculitis and the presence of anti-Ro/La--with a comparatively mild renal pathology. The presence of high titres of RF in SB correlated with the absence of the adequate therapy, the presence of hypergammaglobulinemic purpura, weakly pronounced local manifestations; but a decrease in the titres of RF and an increase in the titres of ANF; anti-nDNA, anti-DNP in young patients with SD proved to be a prognostically unfavourable factor. The presence of CIC was associated with hypergammaglobulinemic purpura and affection of the eyes, and hypocomplementemia--with affection of the kidneys, joints and salivary glands in patients with SD.

Subjective disease experience in Sjögren's syndrome. A computerized discrimination analysis.

A study was made of the ability of a computerized discrimination analysis to distinguish between primary or secondary Sjögren's syndrome on the one hand and, on the other, various rheumatic diseases which may be, but in this study were not, complicated by Sjögren's syndrome. The analysis was based on a questionnaire including 76 two- or three-scale items. Among these 76 questions, five with a maximum potential for distinguishing between various subgroups were selected and obviously represent the questions for the physician to ask when taking the patient's history. As shown by classification matrix tables, computerized analysis of questionnaires might represent a useful way to assess the prevalence of clinical cases of Sjögren's syndrome and to aid health care administrators in assessing the extent of the Sjögren's syndrome problem. In contrast, manual analysis of patients' graded answers did not provide any simple or practicable method for the diagnostic work-up of cases. Therefore it seems that subjective symptoms should not be included in the diagnostic criteria for Sjögren's syndrome, which in clinical work should be based on objective evidence alone.

[Sjögren's syndrome in systemic scleroderma]. / Sindrom Shegrena pri sistemnoi sklerodermii.

Combined investigation of 43 patients with sclerodermia systematica (SSD) was conducted to detect Sjogren's syndrome (SS) and to study SSD association with SS. Four groups of patients were defined: with marked SS (10), primary SS (12), probable SS (13), and without SS manifestations (8). Comparative characterization has shown that SS is detected slightly more frequently in a chronic course of SSD, combines with manifestations of sclerodactyly, telangiectasia, calcinosis, however esophageal hypomobility, indurative skin changes and contractures which are typical of SSD, occur less frequently than in SSD patients without SS. SSD association with SS is characterized by polyarthralgia, arthritis, marked Raynaud's syndrome, the frequency and rather high RF titers, the detection of anti-Ro and anti-La, and a significant rise of the level of circulating immune complexes determined by SS influence. SS manifestations in SSD are retention pains in the parotid glands, signs of clearness on sialograms, periductal sclerosis in the form of rings in morphological investigation of the parotid glands.

[A positive effect of combined therapy (plasmapheresis in combination with pulse therapy) on the course of cryoglobulinemic glomerulonephritis and ulcerative-necrotic vasculitis in patients with Sjögren's disease]. / Polozhitel'noe vozdeistvie kombinirovannoi terapii (plazmaferez v sochetanii s pul's-terapiei na techenie krioglobulinemicheskogo glomerulonefrita i iazvenno-nekroticheksogo vaskulita u bol'noi bolezn'iu Shegrena.

The authors provided evidence of a positive effect of combined therapy (plasmapheresis in combination with pulse-therapy) on a course of cryoglobulinemic glomerulonephritis and ulcerative-necrotic vasculitis in patients with Sjögren's disease (SD). It was shown that plasmapheresis with double plasma filtration considerably decreased the cryoglobulin level in the blood whereas the level of alpha 2-globulins, C-P, RF and CIC did not change 16 hrs after the sessions of plasmapheresis. These indices significantly decreased after pulse-therapy with high doses of 6-methylprednisolone and cyclophosphamide following plasmapheresis. Combined therapy resulted in a complete epithelization of leg ulcers, regression of the symptoms of renal failure. Clinical improvement correlated with a decrease in indices of the inflammatory and immunological activity and with an increase in the cutaneous blood flow. Combined therapy can be an effective therapeutic method of SD complicated by cryoglobulinemic glomerulonephritis and ulcerative-necrotic vasculitis.

[Antinuclear antibodies in Sjögren's disease and syndrome]. / Antinuklearnye antitela pri bolezni i sindrome Shegrena.

A study was made of the presence of antinuclear antibodies to Ro, La, nDNA, RNP and Sm in 18 patients with Sjögren's disease (SD) and in 13 patients with Sjögren's syndrome (SS). Anti-Ro and/or anti-La were revealed in 12 SD patients (67%) and in 3 SS patients (23%, P less than 0.05) only. Anti-nDNA and anti-RNP were detected in 39 and 15% of the SS patients respectively and were undetectable in the SD patients. Anti-Ro and/or anti-La were detected in all SD patients, III degree activity, and associated with the presence of Raynaud's syndrome, recurring nonerosive arthritis and the absence of adequate therapy. Anti-Ro only were detected in 44% of the SD patients only and in none of the SS patients. Higher values of ESR, RF and CIC were revealed in the SD patients with anti-La and especially anti-Ro.