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ID PMID Title PublicationDate abstract
1711713 [Serum beta 2-microglobulin and HLA alloantigens in primary Gougerot-Sjögren syndrome. A 1991 May The relationships between some allo-antigens of the HLA system and beta 2-microglobulin (beta 2m) serum level were examined in a group of 24 subjects with primitive Sjögren's syndrome (pSS). While the beta 2m serum level of all the patients with pSS were higher at the limits of significance (p congruent to 0.05), compared to the values of the 14 control subjects, the division of the patients into two sub-groups of 14 and 10 subjects, according to the presence or absence of the haplotypes DR2 and/or DR3, pointed up a beta 2m serum level which was significantly higher in the first compared to the second (p less than 0.02) and to the group of normal subjects (p less than 0.01). Among the individual haplotypes studied, only the DR3 was observed with a significantly greater frequency (p less than 0.01) in the patients compared to the control group. The haplotype DR3 and also the B8, although at a lesser level, were found to be correlated with a high value of the serum beta 2m: p less than 0.004 and p less than 0.05 respectively. A similar association was not found for the DR2 and DRW52 specificities.
2071745 Lymphocyte response to staphylococcal enterotoxin B in patients with annular erythema asso 1991 Feb Lymphocyte response to staphylococcal enterotoxin B (SEB) was analysed in 11 cases with Sjögren syndrome (SjS) who developed annular erythema during the course. Increased lymphocyte response against SEB was observed on day 5 in those patients who developed annular erythema at the time of examination. A similar reaction pattern was observed when Con A and PWM, but not protein A, were used as the mitogen. No significant lymphocyte response was observed in the patients in the inactive stage without annular erythema or in normal controls. Furthermore, peripheral blood lymphocytes express LFA-1 after SEB stimulation. These results suggest that lymphocytes from SjS patients react to various types of antigenic or mitogenic stimulation and that they express cell adhesion molecules, especially in patients with active annular erythema.
2803324 A new and highly sensitive immunohistologic diagnostic criterion for Sjögren's syndrome. 1989 Oct Immunohistologic investigation of the sublabial salivary glands of 65 surgical patients who were free of systemic disease (control) and 19 patients with Sjögren's syndrome resulted in a new diagnostic criterion for SS that was more disease-specific than the widely used lymphocytic focus score criterion. Our bivariate classification criterion, which is based on the percentages of IgA- and IgG-containing plasma cells, has a specificity of 95.4% and a sensitivity of 100%, while its overall percentage of misclassification is 3.6%. This immunohistologic diagnostic criterion results in a reduction of the percentage of false-positive diagnoses from 9% to 4.6%.
2694285 [Myelopathy, polymyositis and systemic manifestations associated with the HTLV-I virus]. 1989 A case of HTLV-I associated myelopathy in a 51 year-old Haitian woman is reported. MRI showed high signals in the cerebral white matter on T2-weighted images. There also was clinical and electrophysiological evidence of myositis, and a biopsy of the quadriceps muscle showed dense inflammatory infiltrates surrounding several small perimysial blood vessels. The virus was not demonstrated in the muscle. The presence of several systemic abnormalities (polyclonal gammapathy, circulating immune complexes, Sjögren's syndrome) and the vasculitis suggest an immunopathological mechanism for this HTLV-I associated myositis.
3072138 Immunohistochemical identification of infiltrating mononuclear cells in tubulointerstitial 1988 Dec Using monoclonal antibodies, immunoperoxidase analysis was performed on eight renal biopsy samples obtained from patients with Sjögren's syndrome-associated tubulointerstitial nephritis. In all cases the interstitial infiltrates were predominantly CD5-positive T-cells (mean 66%), whereas both B-cell (CD19-positive) and monocyte (CD15-positive) populations participated to a lesser degree. CD16-positive NK/K cells were rarely encountered. The CD4/CD8 ratio was consistently higher than 1.0 (mean 2.08). The lymphocytes which invaded the tubular epithelial cells to present a feature of tubulitis were CD8-positive, suggesting that they were cytotoxic T-cells. These results were in general accord with those obtained in the salivary glands of patients with Sjögren's syndrome. It was thus concluded that the same immunological process was probably operative in the renal tubulointerstitial tissue as in the salivary glands to induce the characteristic tissue changes of Sjögren's syndrome.
3165742 Chronic myelomonocytic leukemia and primary Sjögren's syndrome. 1988 Mar Although associations of Sjögren's syndrome (SS) with malignant affections are well known, an association with chronic myelomonocytic leukemia (CMML) is infrequent. Our report concerns an 80-year-old woman with chronic polyarthrosis, hospitalized for an anemic syndrome, who also complained of buccal dryness. Clinical examination showed simply parotid swelling and discrete splenomegaly. The diagnosis of SS appeared to be primary. The hemogram, myelogram and bone biopsy indicated CMML. During SS, the possibility of occurrence of a lymphoproliferative syndrome is well documented, and other malignant affections are much less likely to be found in the absence of immunosuppressive treatment. The particular case of SS and associated CMML detected at the same time suggests either the favorable role of monocytic proliferation on immunity or a stem clonal anomaly affecting monocytes and B lymphocytes.
3261539 Complete congenital heart block followed by anti-Ro/SSA in adult life. Studies of an infor 1988 Feb A family is described in which an asymptomatic woman gave birth to a male child with complete congenital heart block. Precipitating antibodies to the Ro/SSA antigen have developed in this child, now aged 33, who is clinically well. In the mother, features of both systemic lupus erythematosus and Sjögren's syndrome developed 26 years after the birth of her son. Her serum currently has precipitating antibodies to both the Ro/SSA and La/SSB antigens. The presence of anti-Ro/SSA in the child with complete congenital heart block and the extremely long delay of 26 years between the delivery of the child and the development of clinical disease in the mother are discussed in light of investigators' developing knowledge of both neonatal and adult systemic lupus erythematosus.
3500017 Lymphocyte subpopulations in bronchoalveolar lavage in Sjögren's syndrome. Evidence for a 1987 Dec We initiated this study to determine the cellular composition and T-lymphocyte subpopulations of fluid from bronchoalveolar lavage from 15 patients with primary Sjögren's syndrome (1SS), six patients with secondary Sjögren's syndrome associated with primary biliary cirrhosis (2SS-PBC), eight patients with secondary Sjögren's syndrome associated with collagen-vascular diseases (2SS-CVD), and 12 normal subjects. All were nonsmokers who were free of clinical pulmonary symptoms and had normal findings on chest roentgenograms. Lymphocyte subsets were identified by mouse monoclonal antibodies that were specific for T-cells, helper/inducer, and suppressor/cytotoxic (namely, OKT3, OKT4, and OKT8). Patients with 1SS, patients with 2SS-PBC, and patients with 2SS-CVD had a significantly increased percentage of lymphocytes in fluid from bronchoalveolar lavage (respectively, 21.6 +/- 3.7 percent, 24.3 +/- 6.1 percent, and 25.6 +/- 3.9 percent) compared with the normal value of control subjects (9.9 +/- 1.5 percent). In addition, two of the 15 patients with 1SS and five of the eight patients with 2SS-CVD demonstrated an increased percentage of alveolar neutrophils. The predominant T-cell subset in patients with 1SS was T4+, and the mean T4:T8 ratio was normal. The percentage of T4+ cells was increased in patients with 2 SS-PBC, resulting in an increased T4:T8 ratio. In contrast, patients with 2 SS-CVD demonstrated a markedly increased percentage of T8+ cells, reflected by a shift in the T4:T8 ratio which was inverted. Patients with Sjögren's syndrome and with neutrophilia on bronchoalveolar lavage had a marked expansion of the T8+ lymphocyte subpopulation, where as patients with Sjögren's syndrome and with pure lymphocytosis on bronchoalveolar lavage showed predominantly T4+ cells. In addition, we found a strong positive correlation between the number of neutrophils and the number of T8+ cells in bronchoalveolar lavage from patients with Sjögren's syndrome (r = 0.74; p less than 0.05). Until the functional activities of OKT4+ and OKT8+ cells are better defined, the role that these cells play in the pathogenesis of pulmonary disease in Sjögren's syndrome remains unclear.
2544327 Neuropsychiatric abnormalities in primary Sjögren's syndrome. 1989 Mar Neuropsychiatric abnormalities were searched for in 52 patients with primary Sjögren's syndrome (SS). 40 patients were evaluated with a detailed neurologic history and physical examination and determination of terminal latencies and nerve conduction velocities of all limbs. Nineteen of them, plus another 12 primary SS patients were evaluated for hostility structure and psychiatric symptoms, using the Hostility and Direction of Hostility Questionnaire (HDHQ) and the Symptoms Check List-90R (SCL-90R) respectively and the results were compared with those of 33 healthy women and 41 cancer patients. Three patients had face numbness and/or hypesthesia indicating trigeminal involvement. Ten had mild sensory or mixed neuropathy of the glove-stocking type. None of our patients volunteered peripheral nervous system (PNS) symptoms nor did we detect central nervous system (CNS) involvement in any of them. One patient with purpura, glomerulonephritis and cryoglobulinemia presented with severe mononeuritis multiplex. High levels of introverted hostility were reported by SS patients in relation to the other two groups. In addition, higher scores on paranoid ideation, somatization and obsessive compulsiveness were found in SS patients compared to the rest but no correlation was found between psychiatric symptomatology and neurologic abnormalities. It is suggested that PNS disease is relatively common and benign in most primary SS patients, psychiatric disorders, sometimes serious, are also common, but CNS involvement must be rather rare.
3359647 Sjögren's syndrome: clinical, cytological, histological and colposcopic aspects in women. 1988 Sjögren's syndrome, characterized by a progressive alteration of the exocrine glands which ultimately results in their atrophy, has the highest incidence among females. The main etiopathogenetic mechanism is autoimmunological. The symptomatology is made up of symptoms depending on an altered glandular secretion and mucosal dryness. Dyspareunia and pruritus, due to vaginal and vulvar dryness, are quite common symptoms. Few studies have considered the clinical and histological consequences of Sjögren's syndrome in the external female genitalia. In the present study 26 women (mean age 46 years) affected by Sjögren's syndrome were examined by means of a series of tests including gynecological examination exam, colposcopic inspection and cervical biopsy. All the patients showed a particular dryness a series of tests including gynecological examination failed to evidence malign cells and, in 15% of the subjects, showed an estrogenic insufficiency in various degrees. The colposcopic inspection revealed dystrophic processes resulting in the atrophy of the cervico-vaginal mucosa in 50% of the cases. The histological findings of the cervical biopsies evidenced the presence of a chronic cervicitis in 10% of the cases.
1961346 Subacute cutaneous lupus associated with Hashimoto's thyroiditis. 1991 Aug Subacute cutaneous lupus erythematosus is a widespread, non-scarring, photosensitive form of histologically specific cutaneous LE. These patients frequently have mild systemic illness marked by musculoskeletal complaints and characteristic serologic abnormalities. Hashimoto's thyroiditis coexists with other diseases of presumed autoimmune nature, including systemic lupus erythematosus. The association between subacute lupus and Hashimoto's disease has not been described. We describe here a patient with Hashimoto's thyroiditis and Sjögren's syndrome who developed subacute cutaneous lupus two years later.
2327235 Nervous system manifestations in Sjögren's syndrome. 1990 Feb Central and peripheral manifestations of the nervous system were evaluated in 48 Sjögren's syndrome patients. Fifty-six percent of the patients had neurological disturbances. The most common manifestations were entrapment neuropathies (19%) and polyneuropathy (15%). Electrophysiological tests gave further evidence of subclinical nervous system involvement in Sjögren's syndrome: electroencephalography (EEG) was abnormal in 48%, and visual evoked potentials (VEP) in 12% of patients tested. To find possible neuropsychiatric abnormalities, the Minnesota Multiphasic Personality Inventory was applied, and 33/43 patients were found to have psychiatric symptoms. The most frequent were depressive symptoms. In 44% of the patients there was additional evidence of extraglandular involvement or autoimmune disorders. No correlation could be found between the groups of patients with or without neurological disturbances in relation to simultaneous occurrence of associated disorders. It is suggested that nervous system involvement in Sjögren's syndrome reflects the pathogenetic consequences of Sjögren's syndrome alone, and not those of associated autoimmune diseases of extraglandular disorders.
2661073 Registration procedures and exchange of data in the Sjögren's syndrome research team with 1989 Mar The management of patients with Sjögren's syndrome and research on diagnostic procedures and treatment modalities is to a great extent a co-operation procedure between specialties such as rheumatology, ophthalmology and oral medicine. Efficient registration and handling of data between those disciplines is of the utmost importance for the evaluation of diagnostic criteria and therapeutic measures. Routines for the handling of pertinent data between an EDB center and different clinics are suggested in this article. For the efficient registration a package of forms is also demonstrated. This package contains one main chart and separate charts for registration of detailed information from each discipline involved.
3287567 Parotitis due to anaerobic bacteria. 1988 Mar Although Staphylococcus aureus remains the pathogen most commonly implicated in acute suppurative parotitis, the pathogenic role of gram-negative facultative anaerobic bacteria and strict anaerobic organisms in this disease is becoming increasingly recognized. This report describes a case of parotitis due to Bacteroides disiens in an elderly woman with Sjögren's syndrome. Literature reports on seven additional cases of suppurative parotitis due to anaerobic bacteria are reviewed. Initial therapy of acute suppurative parotitis should include coverage for S. aureus and, in a very ill patient, coverage of gram-negative facultative organisms with antibiotics such as cloxacillin and an aminoglycoside. A failure to respond clinically to such a regimen or isolation of anaerobic bacteria should lead to the consideration of the addition of clindamycin or penicillin.
1857992 [Evaluation of two quantitative tests for salivary secretion--the chewing gum test and the 1991 Feb A cooperative multicenter study was performed to evaluate two salivary secretion methods-the chewing gum test and the Saxon test by a crossover method. Twenty healthy volunteers and 27 patients with Sjögren's syndrome were examined. In healthy controls the amount of saliva collected per minute was larger in the Saxon test. Both tests revealed a significant decrease of salivary secretion in patients with Sjögren's syndrome, but the amount of saliva per minute was also larger in Saxon test. By repeated trials of both tests a notable increase of saliva was always observed at the second trial. Since this phenomenon may be due to the learning effect of the tests, one should perform at least one practice before starting formal tests. Saxon test could be performed in a patient having dental prostheses who was unable to masticate chewing gum. The Saxon test, thus, may be an efficient method, to evaluate the salivary secretory function.
2913935 The prevalence of primary Sjögren's syndrome in a multiple sclerosis population. 1989 Jan A central nervous system illness closely resembling multiple sclerosis has been described in patients with primary Sjögren's syndrome. From these reports, the estimated prevalence of this syndrome could be similar to that of multiple sclerosis in a high-frequency area. We evaluated 192 consecutive patients in our multiple sclerosis clinic to determine if such cases had gone unrecognized. We did not find any patients with clinical or serological evidence of primary Sjögren's syndrome. We conclude that it is rare for neurological manifestations of Sjögren's syndrome to mimic multiple sclerosis in our population.
3248213 Lymphocytic interstitial pneumonitis in association with Sjogren's syndrome. 1988 Jul We describe a patient with Sjogren's syndrome who developed what appeared to be fibrosing alveolitis. Transbronchial lung biopsy indicated lymphocytic interstitial pneumonitis (LIP), rather than fibrosing alveolitis, which was treated successfully with steroids. Both conditions are well recognized in association with Sjogren's syndrome and the distinction is important in view of the good response to therapy of LIP.
3577702 Relation between blink frequency and break-up time? 1987 Feb A possible correlation between break-up time (BUT) and blink frequency was investigated in 32 healthy persons (17 females and 15 males) and in 29 patients suffering from primary Sjögren's syndrome (27 females and 2 males). A significant negative correlation, was found between BUT and blink frequency. In persons with high BUT, the blink frequency is low, probably just sufficient to prevent spontaneous tear breaks. In persons with low BUT, i.e. the Sjögren patients, the blink frequency is high, and tear fluid breaks are mostly prevented.
2491640 Polyarthritis in MRL-lpr/lpr mice: mouse type II collagen is antigenic but not arthritogen 1989 In addition to a lupus-like syndrome and massive T cell proliferation, MRL-lpr/lpr(MRL/l) mice develop an arthritic process very similar serologically and histologically to human rheumatoid arthritis (RA). Recently, we have developed in DBA/1 mice an experimental model of autoimmune arthritis (EAA) which shares clinical features with RA, by injecting homologous type II collagen (CII). In order to investigate the possible relationship between the spontaneous polyarthritis of MRL/l mice and collagen induced EAA, we immunized MRL/l mice with mouse (M) CII. Our findings revealed that the injection of 100 micrograms M-CII in young or old MRL/l mice did not modify the articular pathology which spontaneously develops in non-injected mice. Circulating autoantibodies to native M-CII were found in the sera of immunized young mice but were not detected in non injected or immunized old mice. Conversely, denatured alpha 1 (II) chains or CB peptides derived from M-CII were recognized by most of the MRL/l sera whether mice had been immunized or not. The incidence of positive sera as well as the intensity of the response evaluated by Western blot analysis increased with the age of the mice. Taken together, our data suggest that, even if the injection of homologous CII in MRL/l mice may accelerate the onset of joint pathology, the spontaneous disease arises independently of an autoimmune response against native CII.
2096441 [Agranulocytosis and connectivitis different from Felty's syndrome: 4 cases. Success of cy 1990 Jul Four cases of chronic agranulocytosis in patients with systemic diseases different from Felty's syndrome are reported. Two patients had primary Sjögren's syndrome (confirmed in one, suspected in the other), one had systemic lupus erythematosus and the last patient had an unclassifiable connective tissue disease. Only one severe infection was recorded. The pathophysiological mechanisms involved are discussed. There is a strong suspicion that the cytopenia is of immune origin. Corticosteroid therapy was effective in all four patients. One patient who could not tolerate corticosteroids was successfully treated with cyclosporin A.