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ID PMID Title PublicationDate abstract
8124907 Anti-SSA/Ro and anti-SSB/La antibodies in serum and saliva of patients with Sjogren's synd 1993 Dec Autoantibodies to SSA/Ro and SSB/La antigens may have a pathogenic role in photosensitive skin disease and congenital complete heart block. Since salivary glands are the major target organ in Sjogren's syndrome (SS) we wondered whether these autoantibodies are present in saliva and may be involved in the sicca syndrome. Whole saliva and serum were collected from 15 patients with SS. Elisa analysis disclosed that 8 of the patients had anti-SSA/Ro antibodies, while 6 of them also had anti-SSB/La antibodies. Studies of immunoglobulin classes showed that the sera contained mainly IgG and IgM anti-SSA/Ro or SSB/La antibodies. One serum also contained IgA antibodies. Analysis of the saliva showed that in all positive samples IgG and IgA classes were present, while none of them contained IgM. Elisa and immunoblot analysis of sera and saliva from SLE patients without the sicca syndrome disclosed that both fluids contained anti-Sm antibodies. These findings suggest that the presence of anti-SSA/Ro and anti-SSB/La antibodies in saliva is not a unique phenomenon, characterizing the sicca syndrome. Therefore, their role in the pathogenicity of the Sjogren's syndrome has to be elucidated.
1464870 Raynaud's phenomenon in primary Sjögren's syndrome. Association with other extraglandular 1992 Oct One hundred and four patients with primary Sjögren's syndrome (SS) were evaluated for the presence (29%) or absence of Raynaud's phenomenon (RP). The clinical course of RP was, in general, benign and caused no vascular sequelae. In patients with primary SS having RP, nonerosive arthritis, vasculitis and pulmonary fibrosis were significantly more frequent than in those without RP. Myositis also appeared more frequently associated with RP, but the difference reached statistical significance only when combined in meta-analysis with 2 other comparable series. There were no differences in the autoantibody profiles of the 2 groups.
1399454 Quantitative evaluation of sialograms. 1992 Sep RATIONALE AND OBJECTIVES: The authors developed and evaluated a quantitative analytic method for interpreting clinical sialograms. METHODS: Images were obtained by digital subtraction sialography and transformed into binary form. The duct width of the image was calculated and represented as a normalized histogram. The effects of the volume of contrast medium injected and of the inclination angle of the objects on the histogram were examined. RESULTS: In model studies, the normalized histogram was affected insignificantly by these factors. Clinical sialograms of 18 patients with normal results, 12 patients with parotitis, and 5 patients with Sjögren syndrome were preliminarily analyzed by the histogram method and four representative parameters of the histogram. Discriminant analysis showed a relatively high correct-predictive rate in the distinction between patients with normal and abnormal results. CONCLUSIONS: This method reduces the effect of observer variation in diagnosing sialograms, and improves diagnostic accuracy for assessing the presence of inflammatory diseases of the parotid gland.
1351942 Lack of deletion of complement C4 and steroid 21-hydroxylase genes in Japanese patients wi 1992 May A null allele at C4A (C4AQO) is associated with primary Sjögren's syndrome (SS) in Japanese. Since a deletion of the C4A and CyP21A genes is reported to account for C4AQO in patients with systemic lupus erythematosus (SLE) in Caucasians, we studied the restriction fragment length polymorphism (RFLP) of genomic DNA to determine whether similar deletions of the C4A and CyP21A genes occur in Japanese patients with SS. Patients with C4AQO did not show the extensive deletion of C4A and CyP21A, which would be recognized by the appearance of 8.5 kb/HindIII and 6.4 kb/TaqI fragments hybridizing with a C4 probe. It is yet to be shown whether the lack of expression of C4A genes in Japanese patients with primary SS is due to point mutations, or to small deletions or insertions that were not detected by the RFLP approach.
1285317 Levels of CD5+ B cells are not increased in probands or relatives in a family study of pri 1992 Levels of CD5+ B lymphocytes were assayed in a large family study of Primary Sjögren's syndrome. There was no significant difference in CD5 expression by index cases or their relatives when compared to controls. No association between CD5 expression, serological abnormalities or HLA haplotype was found and, furthermore, no evidence of linkage with HLA was observed. There was, however, variation in the expression of CD5+ B cells between the families. Levels in spouses were lower and reached statistical significance. The role for genetic and environmental factors influencing CD5 expression is discussed. Any genetic influence does not appear to involve the HLA region or genes in linkage disequilibrium.
7722088 Association of the acral type of pustular psoriasis, Sjögren's syndrome, systemic lupus e 1995 Feb We describe a case of a 53-year-old Japanese female suffering from Sjögren's syndrome, systemic lupus erythematosus, and Hashimoto's thyroiditis who developed pustules, erythema, and erosions on her fingers and toes. The histological specimen showed psoriatic changes. Indirect immunofluorescent study using anti-human IL (interleukin)-8 antibody produced positive staining patterns in the lesional epidermis. These findings suggested the diagnosis of acral pustular psoriasis. Diaminodiphenylsulfone at 75 mg orally daily for 20 days and the application of 0.12% betamethasone valerate ointment led to gradual improvement.
1479116 A case of cytophagic histiocytic panniculitis with sicca symptoms and lupus nephritis. 1992 Sep A 48-year-old Japanese woman presented with many subcutaneous nodules. The skin was purplish in color and tender; the nodules were scattered over the entire surface. Histological findings of biopsy specimens from the nodules indicated septal panniculitis comprised of histiocyte and/or macrophage infiltrates, often with erythro- and/or leukophagocytosis. Phagocytic cells were OKM1 (CD11b), MT1 (CD43), LeuM3 (CD14), and histiocyte antigen positive, indicating the presence of histiocytes and/or macrophages. The patient had sicca symptoms, positive homogenous, speckled pattern ANA (x320), and diffuse proliferative lupus nephritis.
8948618 Detection of Epstein-Barr virus in salivary gland specimens from Sjögren's syndrome patie 1996 Dec The cause of Sjögren's syndrome remains unclear, but several environmental and genetic factors have been implicated. The Epstein-Barr virus (EBV), among others (e.g., cytomegalovirus, human herpesvirus 6, and retroviruses), has been widely studied in connection with Sjögren's syndrome without conclusive results. To determine the role of EBV infection in patients with Sjögren's syndrome, the presence of EBV deoxyribonucleic acid (DNA) in major and minor salivary gland biopsy specimens was investigated by means of sulfur 35 in situ hybridization and polymerase chain reaction. Additionally, the presence of latent virus proteins EBV latent membrane protein and Epstein-Barr nuclear antigen 2 was analyzed by immunohistochemical methods. Viral DNA, detected by in situ hybridization, was found in 19% of patients with a diagnosis of Sjögren's syndrome and in 3% of controls. All tissues studied were found to be negative for EBV DNA by polymerase chain reaction. EBV latent membrane protein-positive staining was seen in 17% of patients and 22% of control subjects, while Epstein-Barr-positive staining was found in 25% of patients and 39% of controls. The low frequency of EBV DNA detected in the biopsy specimens does not indicate that the virus itself is the cause of Sjögren's syndrome. However, the possibility that the virus acts as a cofactor cannot be ruled out.
8809450 Autoimmune thyroid disease in systemic lupus erythematosus and Sjögren's syndrome. 1996 May Autoimmune thyroid disease is common in patients with either systemic lupus erythematosus (SLE) or Sjögren's syndrome (SS); however, whether the risk is greater than in well matched controls is not clear. Antinuclear antibodies are found in the sera of patients with autoimmune thyroid disease, but there is no evidence of autoantibodies binding antigens characteristic of SLE or SS. Autoantibodies to thyroid antigens and overt autoimmune thyroid disease are common in patients with SLE or SS. However, in SLE patients there is little or no increased risk of autoimmune thyroid disease compared to an age and sex matched population. Patients with SS are likely to be at increased risk of thyroid disease, but those with autoimmune thyroid disease are not at special risk of SS. This difference can be explained if SS is considered to be an autoimmune epitheliitis that targets polarized epithelia, including the thyroid.
7778681 Anion exchanger immunoreactivity in human salivary glands in health and Sjögren's syndrom 1995 Jun Salivary gland ducts play a relevant role in saliva secretion through transport processes. Na(+)-independent chloride-bicarbonate anion exchangers (AE) may be involved in these processes by generating ion fluxes into the salivary secretion. In Sjögren's syndrome, a disorder with gland dysfunction, there might be an impaired expression of AE proteins. Here we study AE immunoreactivities in human salivary glands, both in health and in Sjögren's syndrome. Immunohistochemistry was carried out on salivary glands from normal subjects and patients with Sjögren's syndrome, using two monoclonal antibodies against AE1 and AE2. Normal salivary glands showed AE2 immunoreactivity, which was restricted to the epithelium of the ducts, with no staining at the acini. A strong positivity was seen in the basolateral portion of the striated ducts, while interlobular duct cells showed a discrete positivity at their apical pole. In salivary glands from most of the patients with Sjögren's syndrome, AE2 immunoreactivity was absent in the ducts as well as in the acini. In both normal and diseased salivary glands, AE1 immunoreactivity was only located at the erythrocyte membrane. The recently reported AE0 was discarded because no AE0 message was found in salivary glands by reverse transcription polymerase chain reaction. In conclusion, AE2 immunoreactivity is observed in the ducts of normal salivary glands, particularly in the striated ducts. AE2 immunoreactivity is virtually absent in salivary glands from patients with Sjögren's syndrome, which may reflect either a loss of AE2 after inflammatory atrophy, or a primary defect occurring in the disease.
7839102 Mild sialoadenitis: a common finding in patients with hepatitis C virus infection. 1994 Oct BACKGROUND: Sjögren's syndrome (SS) is an autoimmune disease of presumed viral origin; sialoadenitis has been reported to occur in hepatitis C virus (HCV) infection. METHODS: Lip biopsy specimens were graded in 32 consecutive patients with either HCV-related chronic liver disease or SS. RESULTS: Seventeen of 22 HCV-positive patients had sialoadenitis, although generally mild (15 of 17, grades I-II). Severe inflammation (grades III-IV) was observed in 8 of 10 patients with SS (chi-square = 12.6; P < 0.0005). Moreover, HCV-positive patients with sialoadenitis differed from patients with SS in female sex prevalence (6 of 17 versus 10 of 10; chi-square = 10.9; P = 0.0001) and presence of serum antinuclear autoantibodies (0 of 17 versus 9 of 10; chi-square = 23.0; P < 0.0001). Five of 13 HCV-positive patients and 7 of 8 patients with SS were HLA-DR3-positive (chi-square = 4.9; P < 0.05). CONCLUSIONS: Sialoadenitis of HCV-related liver disease is common but differs from SS with regard to predisposing genetic factors, expression of autoimmune markers, and histopathologic severity.
8480140 More evidence of distinctive features of mixed connective tissue disease. 1993 A comparison of clinical, immunological and HLA-D region antigen features was made between 22 patients with mixed connective tissue disease (MCTD) and 118 patients with systemic lupus erythematosus (SLE), scleroderma or primary Sjögren's syndrome. The MCTD patients had hypergammaglobulinemia more often than did those with SLE and scleroderma, but had less skin ulceration, serositis, nephritis, central nervous system disease and hypocomplementemia than the SLE patients. The frequencies of HLA-DR4 and its Dw4 subtype were significantly increased in MCTD as compared with both the other patient groups and healthy controls. Anti-RNP antibodies and the clinical characteristics together seem to illustrate a disease syndrome which is clinically and genetically distinct and fits with the prevailing concept of MCTD.
1446492 Liquid phase characteristics of bronchoalveolar lavage in primary Sjögren's syndrome. 1992 Dec We have previously reported that alveolitis correlates with clinical, roentgenologic, and functional parameters of pulmonary involvement in primary Sjögren's syndrome (1SS). In the present study, we analyzed the liquid phase characteristics of bronchoalveolar lavage (BAL) in the same 19 patients with 1SS. Our results show that patients with "high alveolitis" (group A, BAL lymphocytes > 15.2 percent) have increased values of total protein, albumin, IgA, and IgG and in their BAL fluid compared with patients with "low alveolitis" (group B) and control subjects. Also interleukin 2 (IL-2) was detected in more "high alveolitis" patients while IgM, IL-2R, and interferon gamma (IFN-gamma) were detected only in this group. There were no differences between the two groups in serum values of all above factors as well as in the presence of rheumatoid factor, extractable nuclear antibodies, and antinuclear antibodies. The increased values of immunoglobulins and cytokines in the BAL fluid of patients with intense alveolitis, in the absence of serum differences, speak for their local production and suggest activation of local immune mechanism.
1407988 Salivary gland involvement in autoimmune thyroiditis, with special reference to the degree 1992 Sep From a total of 63 patients with autoimmune thyroiditis, 19 cases were further investigated to determine the degree of concomitant morphologic and functional salivary gland changes. For comparison, 21 of a total of 28 cases of primary Sjögren's syndrome were also examined. Of the 19 cases of autoimmune thyroiditis, 11 showed various degrees of salivary gland involvement on the basis of an analysis of lower lip salivary gland biopsy specimens, scintigraphy of the parotid, and unstimulated whole sialometry. Six of these cases fulfilled the criteria of primary Sjögren's syndrome. A remarkably high proportion of dark-staining acini was observed in the lower lip biopsy specimens of our patients with thyroiditis (8 of 19, 42%) and less among our patients with primary Sjögren's syndrome (5 of 21, 24%). We conclude that significant involvement of salivary glands may occur in cases of autoimmune thyroiditis, which indicates that common mechanisms may frequently be operative in the development of thyroid and salivary gland immune disease.
8803908 Treatment of primary Sjögren's syndrome with hydroxychloroquine: a retrospective, open-la 1996 Jun OBJECTIVES: To determine whether patients with primary Sjögren's syndrome (SS), diagnosed according to San Diego criteria, had improvement in their laboratory or clinical features during treatment with hydroxychloroquine (6-7 mg/kg/day) for at least two years. METHODS: The study population included 50 consecutive patients with primary SS who were diagnosed according to San Diego criteria, and in whom hydroxychloroquine was suggested as treatment. This group included 10 patients who were early dropouts (side effects or desire not to take antimalarial drugs) and 40 patients who received drugs for at least two years (range 24-48 months). In a subset of SS patients, values for ESR (westergren) and quantitative immunoglobulins were available for comparison. Improvement with therapy was defined as: (a) > or = 20% improvement in variables of tear flow (Schirmer's test I) or corneal integrity (rose Bengal): (b) > or = 20% salivary function (flow rate); and (c) improvement in at least two of the following measures: physicians assessment of global disease activity by > or = 20%, patient assessment of improvement in pain or fatigue by > or = 20%, and ESR improved by > or = 20 mm/hr. RESULTS: In a retrospective study of SS patients who completed the trial, a significant improvement was noted in ocular symptoms (pain and dryness) in patients (55 and 57%) and improved corneal integrity (rose Bengal straining) in 53% of patients. The Schirmer's test was improved by > or = 2 mm/5 minutes in 50% in patients. Improvement was noted in oral symptoms (pain and dryness) in patients (57 and 60%) and salivary flow rate was increased in 82% of patients. In a subset of SS patients evaluated, the ESR improved by > or = 20 mm/hr in 17/32 patients (53%) and quantitative IgG level by > or = 20% in 8/13 patients (61%). Physician global assessment of overall patient status and patient assessment of overall status indicated improvement in over 62% of patients. CONCLUSION: In a retrospective study of patients fulfilling San Diego Criteria for SS, we found: (a) sustained improvement of local symptoms (painful eyes, painful mouth) and improvement of systemic manifestations (arthralgias and myalgias) after treatment with hydroxychloroquine 6-7 mg/kg/day over mean three-year follow-up; (b) laboratory analysis showed a significant improvement in their ESR and their quantitative IgG levels; (c) no significant late toxicity was observed in this study cohort. A prospective study of hydroxychloroquine in patients fulfilling San Diego criteria for SS is indicated.
8871759 Peripheral blood in Sjögren's syndrome does not contain increased levels of T lymphocytes 1996 Patients with Sjögren's syndrome (SS) frequently have anti-Ro/SS-A and anti-La/SS-B autoantibodies. The aim of this study was to investigate if these patients have peripheral blood lymphocytes (PBL) secreting IFN-gamma after short-term cultivation in the presence of Ro/SS-A and La/SS-B antigens. The frequency of PBL secreting IFN-gamma was examined in 12 SS patients and 11 healthy controls. The enzyme-linked immunospot (ELISPOT) assay was performed after 48 hours cultivation of PBL in the presence of recombinant Ro 52 kD protein or recombinant La 48 kD protein. The number of unstimulated IFN-gamma secreting cells in the SS patient group was not significantly different from that of the control group. Moreover, no increase in the number of IFN-gamma secreting cells after Ro/SS-A and La/SS-B stimulation was detected in the two groups. Thus, T cells reactive with the recombinant Ro 52 kD and La 48 kD proteins do not occur with any increased frequency in peripheral blood of SS patients.
8538072 Optic neuropathy and acute transverse myelopathy in primary Sjögren's syndrome. 1995 A case of Sjögren's syndrome with optic neuropathy and acute transverse myelopathy mimicking multiple sclerosis is described. The patient suddenly lost her visual acuity resulting in a permanent visual deficit in spite of the massive steroid therapy given. During the treatment the patient showed dry eye symptoms and was finally diagnosed as having primary Sjögren's syndrome. It is suggested that primary Sjögren's syndrome should be considered in the differential diagnosis of neurologic disorders that resemble multiple sclerosis.
7653941 Sjögren's syndrome: histologic and immunohistochemical study. 1995 Labial salivary gland tissues from 20 patients with primary Sjögren's syndrome (group 1), 26 with the secondary type of the disease (group 2), 16 patients with the incomplete form of the disease and 10 normal controls were examined for the presence of lymphocyte infiltrates, epithelial damages and plasma cell aggregates. Activated T lymphocytes of the helper phenotype predominated in groups 1 and 2. Aberrant epithelial expression of HLA class II molecules was found in the same group of patients, while IgG- and IgM- containing plasma cells were detected.
7979575 The European Community Study Group on diagnostic criteria for Sjögren's syndrome. Sensiti 1994 Oct AIM: To establish a definitive set of diagnostic criteria in a multicentre European study a selected number of oral and ocular tests were performed on a large number of patients with Sjögrens Syndrome (SS) and controls. The diagnostic accuracy of each test for patients with primary and secondary SS and for controls at different ages, was studied. METHODS: Each centre received a clinical chart describing the series of tests to be conducted. The tests included: questionnaires for dry eye and dry mouth symptoms, Schirmer's-I-test (ScT), tear fluid lactoferrin level (TFLL), break-up time (BUT) and rose Bengal score (RBS) for the eye evaluation; unstimulated and stimulated whole saliva collection (UWSC and SWSC), salivary gland scintigraphy (SGS), parotid sialography (PS) and minor salivary gland biopsy (MSGB) for oral involvement. RESULTS: Data from 22 centres and 11 countries was collected on a total of 447 patients with SS (246 with primary SS and 201 with secondary SS) and 246 controls (of whom 113 had a connective tissue disease without SS). Among the ocular symptoms, the feeling of dry eye and 'sand in the eye' were the ones most commonly recorded in patients with SS. Similarly, the feeling of dry mouth, appearing either spontaneously or when the patient was eating or breathing, was the most frequent subjective oral symptom. Among the ocular tests, ScT showed the best balance between sensitivity and specificity (76.9% and 72.4% respectively), while RBS was the most specific test (81.7%). ScT and RBS gave also sufficiently concordant results. TFLL and BUT gave considerably less reliable results, which were not concordant with each other or with the other ocular tests. The quantitative lacrimal tests ScT and TFLL produced significantly different results in elderly controls, while RBS did not. Abnormal results for all of the ocular tests were less marked and less frequent in patients with secondary SS than in those with primary SS. The oral tests (except SWSC) were generally more reliable than the ocular tests in diagnosing SS. In particular, PS was the most specific diagnostic tools (100%), while MSGB (where the presence of at least one inflammatory focus was considered as indicative for the diagnosis) showed a good balance between sensitivity and specificity (82.4% and 86.2%, respectively). The tests showed a good degree of agreement, and, with the exception of UWSC, were not influenced by age. In the oral, as in the ocular tests, abnormal results were less frequent and less marked in patients with secondary SS. CONCLUSIONS: The results clearly show that ScT and RBS (for the eye evaluation), and SGS, PS, MSGB and UWSC (for salivary gland involvement) are the most reliable tests for the diagnosis of SS. The clinician should be aware, however, that the test results may vary depending on the age of the patient and the type of SS (primary or secondary).
8392992 [Possible involvement of a recently discovered human retrovirus in idiopathic immunologic 1993 May Recent studies have suggested that newly-discovered human retroviruses may contribute to the pathogenesis of several immunological diseases. We linked a human intracisternal A-type retroviral particle (HIAP) to systemic autoimmune diseases, such as Sjögren's Syndrome. A-type retroviruses are envelope-deficient, a property that may contribute to their pathobiology and epidemiology. Potential mechanisms by which a defective retrovirus could induce immune dysfunctions are discussed.