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ID PMID Title PublicationDate abstract
1920970 Association of mixed connective tissue disease, Sjögren's syndrome and autoimmune hepatit 1991 May A 51-year-old female was admitted to the hospital due to liver dysfunction. She had suffered from arthralgia, Raynaud's phenomenon, dry mouth and dry eyes. Laboratory data showed positive LE cells and anti-RNP. The diagnosis of mixed connective tissue disease (MCTD) was made according to the criteria of the international symposium on MCTD and Antinuclear Antibodies (August 1986, Tokyo). Liver biopsy showed chronic active hepatitis. The results of sialography, lip biopsy and rose-bengal test were compatible with Sjögren's syndrome (SjS). This is the report of a rare case with MCTD associated with SjS and autoimmune hepatitis.
2696953 Quantitative immunohistologic and histomorphometric diagnostic criteria for Sjögren's syn 1989 Nov Sjögren's syndrome (SS) is a chronic auto-immune exocrinopathy, especially affecting the lacrimal and salivary glands. The aim of this study is to improve the diagnostic possibilities of the sublabial salivary gland (SSG) biopsy. The SSG biopsies of 19 patients with SS and 65 healthy control subjects were used in a quantitative immunohistologic and histomorphometric study. Statistical analysis of the immunohistochemical data resulted in a diagnostic criterion, which is based on the percentages of IgA- and IgG-containing plasma cells. Statistical analysis of 3 immunohistologic and 6 histomorphometric features resulted in a combined immunohistologic and histomorphometric criterion, which is based on 2 immunohistologic parameters (the percentages IgA- and IgG-containing plasma cells) and 3 histomorphometric parameters (the volume percentages of acini, intralobular ducts and diffuse lymphoplasmacytic infiltrate). The immunohistologic diagnostic criterion has a specificity of 95.4%, a sensitivity of 100% and an overall percentage of misclassification of 3.6%. The combined diagnostic criterion has a specificity of 98.5%, a sensitivity of 100% and an overall percentages of misclassification of 1.2%. Furthermore it reduces the number of false positive diagnoses with a factor 6 from 9% to 1.5%.
2614988 [A case of overlap syndrome of PSS, SLE and Sjögren syndrome treated by cyclosporin A: an 1989 Oct A 26-year-old female patient complicated with PSS, SLE and Sjögren was treated by combination of medium doses of corticosteroid and cyclosporin A. On admission, she showed many abnormal laboratory findings such as increased serum gammaglobulin level, high anti-DNA antibody titer and low compliment level. This therapy improved not only these laboratory abnormalities but clinical symptoms including sclerosis of the skin and arthritis, without notable side effect due to either of these drugs for the following 10 months so far. On the other hand we haven't acknowledged the effect to internal involvement yet. Since there is no promising therapy to sclerosis of the PSS skin, it is suggested that cyclosporin A can be an effective agent for PSS and should be further evaluated.
2736828 Evaluation of tear break-up time, Schirmer's-I test and rose bengal staining as confirmato 1989 Mar The tear break-up time (BUT), Schirmer's I test (S-I) and slit lamp examination after rose Bengal staining (RBS) are simple tests for evaluation of keratoconjunctivitis sicca (KCS). Discrepancies, however, regarding the range of normal values and the specificity and sensitivity of these methods prompted us to reevaluate them in 81 primary Sjögren's syndrome patients (Ss) and 276 normal volunteers. In normal volunteers, BUT values ranged from 4 sec. to 32 sec. (means +/- SD: 13 +/- 6 sec.) and in primary Ss from 1 sec. to 15 sec. (means +/- SD: 7 +/- 3 sec.). Ninety five percent (95%) of the values from primary Ss patients were below 12 sec. Forty two percent (42%) of the values obtained from normal individuals fall within this range. Schirmer's I test values in normal individuals ranged from 0 mm/5 min. to 30 mm/5 min. (means +/- SD: 10 +/- 9 mm) and in primary Ss patients from 0 mm/5 min. to 30 mm/5 min. (means +/- SD: 4 +/- 7 mm.). Ninety five percent (95%) of the primary Ss patients' values were below 19 mm/5 min. Sixty eight percent (68%) of the normal individual values fall into this range. Rose Bengal staining values ranged in normals from 0 to 7 (means +/- SD: 1 +/- 1) and in primary Ss patients from 1 to 8 (means +/- SD: 5 +/- 2). Ninety five percent (95%) of the primary Ss patients' values were above 2. Only 7% of normal individual values fall into this range. From these data we conclude that rose Bengal staining is the best test for evaluation of keratoconjunctivitis sicca.
2525445 Studies on the specificity of the immunohistopathological changes of the salivary glands i 1989 Mar Recently focal lip salivary gland lymphocytic infiltrates, identical to those found in Sjögren's syndrome, has been described in myasthenia gravis, bone marrow transplant recipients and various connective tissue diseases, in the latter conditions without relation to the salivary gland function. In the bone marrow transplant recipients, the lymphocytic infiltrates were shown to be potentially reversible. The findings are summarized, the relation between histopathological alterations and organ functions and the diagnostic specificity of focal lymphocytic infiltrates in lip salivary glands are discussed.
2856043 Antibodies to Epstein-Barr virus and cytomegalovirus in primary Sjogren's syndrome. 1988 Sera from 28 patients with Primary Sjögren's Syndrome (SS) and 20 healthy subjects were tested for the presence of antibodies to Epstein-Barr Virus (EBV) and Cytomegalovirus (CMV). No difference was noted in the mean titre of anti-CMV antibodies in patients and in controls. For what concerns EBV, the mean titre of anti-EBNA antibodies was significantly higher in SS patients than in normals (p less than 0.001); moreover, within the SS population, titres were higher in SSB-positive than in SSB-negative patients (p less than 0.05). These results, together with the fact that anti-SSB antibodies (characteristic of Sjögren's Syndrome) recognize also two EBV-encoded RNAs, strengthen the hypothesis that EBV is involved in the pathogenesis of SS.
2359071 Raynaud's phenomenon in primary Sjögren's syndrome. 1990 May Our study describes the prevalence, clinical picture, course and sequelae of Raynaud's phenomenon in patients with primary Sjögren's syndrome. Furthermore, our study compares the clinical, serologic and immunogenetic profile of patients with Raynaud's phenomenon versus those without Raynaud's phenomenon. It seems that Raynaud's phenomenon is a common manifestation (33%) in patients with primary Sjögren's syndrome, precedes sicca manifestations in 42% of patients and follows a pleomorphic course; in some patients (14%) it disappears during the course of Sjögren's syndrome, in others (30%) the frequency of attacks decreases while in a 3rd group (56%) it remains the same. The local clinical sequelae of Raynaud's phenomenon in patients with primary Sjögren's syndrome are swollen hands and evidence of small, soft tissue calcifications on radiographs. Digital pulp ulcers, sclerodactyly or periungal telengiectases are not seen. It seems however, that patients with primary Sjögren's syndrome and Raynaud's phenomenon develop glomerulonephritis, myositis and peripheral neuropathy more often than patients without Raynaud's phenomenon. These differences however, had no statistical significance. Finally, the autoantibody profile is similar in patients with and without Raynaud's phenomenon, while patients without Raynaud's phenomenon and primary Sjögren's syndrome showed a negative but not statistically significant association with the HLA-DR4 alloantigen.
3473634 Effects of immunosuppressive drugs on the morphology of the microvilli in Sjögren's syndr 1986 Electron microscopic study of conjunctiva in two cases of Sjögren's syndrome showed decrease in height and number of microvilli and lack of branching. Following immunosuppressive treatment, an increase in height, number and branching of conjunctival microvilli was observed. Notwithstanding the lack of influence on tear secretion, immunosuppressive drugs are of benefit in the treatment of ocular manifestations of Sjögren's syndrome. The increase in number, height and branching of microvilli enlarge the surface of the conjunctiva and thus improve the efficacy of artificial tears.
1812449 Relationship between sialographic findings of parotid glands and histopathologic finding o 1991 Dec We investigated a relationship between sialographic findings of parotid glands and histopathologic changes of labial glands, in relation to Sjögren's syndrome. We examined 107 patients, suspected to have Sjögren's syndrome because of dry mouth and dry eyes, in whom no other autoimmune diseases were diagnosed. Parotid sialography identified 37 patients with punctate, globular, cavitary, or destructive sialectasia (S-positive group). These sialographic findings were not seen in the other 70 patients (S-negative group). Among these patients, 54 demonstrated periductal lymphocytic infiltration in the labial glands (H-positive group), whereas the other 53 patients did not demonstrate this histologic finding (H-negative group). Significant differences in stimulated parotid salivary flow rate and incidence of keratoconjunctivitis sicca were observed between patients in the S-positive and the S-negative groups. Differences in the salivary flow rate and the incidence of keratoconjunctivitis sicca were also seen between patients in the H-positive and the H-negative groups, although the differences were not statistically significant. The rate of incidence of serum rheumatoid factor, anti-SS-A, and anti-SS-B antibodies were significantly higher in the S-positive and the H-positive groups than in their negative counterparts.
1753714 Sialoadenitis in experimental graft-versus-host disease. An animal model of Sjögren's syn 1991 Dec Using light microscopic, immunohistochemical, and ultrastructural techniques, we studied sialoadenitis in mice with chronic graft-versus-host disease (GVHD). To induce chronic GVHD, DBA/2 spleen cells were injected into nonirradiated (C57BL/6 x DBA/2)F1 mice. By light and electron microscopy, the submandibular gland lesion was characterized by mononuclear cell infiltration around the interlobular ducts, often with parenchymal destruction. Immunohistochemically, T cells predominated. The ratio of CD4+ to CD8+ T cells ranged from 1.9:1 to 6:1, with an average of 3.6:1. Lesser numbers of B cells, plasma cells, and macrophages were also present. Histologically, lymphocytic sialoadenitis in chronic GVHD mice was very similar to that reported in patients with chronic GVHD and Sjögren's syndrome.
2077152 [Six cases of Sjögren's syndrome with lichen mucosae--studies on the histopathology of sa 1990 Nov A case of a 54-year old man with lichen mucosae and Sjögren's syndrome was reported. Clinical and histopathological analysis was made on the presence of sicca features in 6 patients with lichen mucosae. All patients complained either xerophthalmia or xerostomia and 5 out of 6 cases showed specific lymphocytic foci seen in Sjögren's syndrome in addition to lichenoid tissue reaction in the specimen obtained from buccal mucosae. Objective keratoconjunctivitis sicca was demonstrated in 3 cases. Immunologically, abnormal laboratory findings were observed in 5 cases (4 cases with a positive ANA and 3 cases with a positive anti microsome antibody). These findings suggest that lichen mucosae might develop in a close association with Sjögren's syndrome and relationship between these two disease was discussed in this article.
3177432 Treatment of primary Sjögren's syndrome with hydroxychloroquine. 1988 Oct 14 Sjögren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of the salivary/lacrimal glands, autoantibody production, and polyclonal hyperglobulinemia. In view of the efficacy and relative safety of hydroxychloroquine in other autoimmune disorders, the potential benefit of hydroxychloroquine (200 mg per day for 12 months) in 10 patients with Sjögren's syndrome was evaluated. Changes in levels of total immunoglobulin, antibody against Sjögren's syndrome-associated antigen B, rheumatoid factor, and in vitro production of immunoglobulin in the serum were evaluated. For comparison, 10 patients matched according to age and sex, who did not receive hydroxychloroquine were studied. In the hydroxychloroquine-treated group, the following observations were made: (1) significantly decreased total immunoglobulin G (IgG) and IgA levels with little change in IgM levels; (2) significant decrease in IgA-rheumatoid factor with a smaller decrease in IgM-rheumatoid factor; (3) decreased IgG anti-Sjögren's syndrome-associated antigen B autoantibody; and (4) decreased erythrocyte sedimentation rate and increased hemoglobin level. Further, a specific idiotype present on their rheumatoid factor (defined by monoclonal antibody 17-109) was significantly decreased, with disappearance of detectable circulating paraprotein in two hydroxychloroquine-treated patients. Finally, rheumatoid factor production in vitro by lymphocytes from hydroxychloroquine-treated patients using a T cell-dependent mitogen was significantly decreased. These results suggest that hydroxychloroquine modulates lymphoproliferation in patients with Sjögren's syndrome and may prevent progression to extraglandular sites of neoplastic transformation.
3252807 Two patients illustrating lymphoma transition and response to therapy in Sjögren's syndro 1988 Apr Two patients with Sjögren's syndrome (SS) who subsequently developed malignant B-cell lymphomas are reported in detail. The first patient had both benign- and malignant-appearing lymphoid infiltrates on the same submandibular gland specimen and was successfully treated with combined chemotherapy and irradiation. The second patient developed cutaneous lymphoid infiltrates difficult to diagnose by light microscopy but containing a monoclonal IgM-Kappa population revealed by immunoperoxidase staining and immunoglobulin gene rearrangement studies. Her lesions resolved rapidly and completely on cyclophosphamide, recurred rapidly when this drug was discontinued, and resolved again on a second course of cyclophosphamide which is currently maintained at 50 mg daily. Both patients are doing well without recurrence two and three years after initial treatment. This clinical experience is presented to emphasize: (1) the clinical use of molecular biologic techniques to define the earliest appearance of malignant transformation in Sjögren's syndrome, and (2) the successful outcome that can be achieved with prompt institution of appropriate treatment. The phenomenon of lymphoma development in SS is discussed with regard to immunoregulatory abnormalities predisposing to malignancy in the setting of autoimmune disease.
3260694 [Clinical, cytological and immunological characteristics of the effect of ENKAD on the mou 1988 A study of the clinical efficacy and the immunomodulating and antiinflammatory effect of ENCAD was conducted in 20 patients with Sjogren's disease (SD) of various degrees of activity of oral mucosa involvement and in a group of 23 healthy controls, with a saline solution as placebo. ENCAD was shown to lessen the expression of the clinical symptoms of xerostomia in both groups of a low and a moderate and high degrees of local activity of an inflammatory process. A tendency to the normalization of regeneration and desquamation in the oral mucosa epithelium was observed against a background of the reduction of an inflammatory reaction. ENCAD produced an immuno modulating effect lowering the blood level of theophylline-resistant and raising the level of theophylline-sensitive T-lymphocytes.
3493738 Effect of cyclosporin A (CyA) on the immunopathological lesion of the labial minor salivar 1987 Feb Labial minor salivary gland biopsy specimens from 14 patients with Sjögren's syndrome treated either with cyclosporin A (CyA) or placebo (5 mg/kg body weight day for six months) were studied to determine T lymphocyte subsets and HLA-DR antigen expression using the avidin-biotin-peroxidase technique. In all CyA treated patients we observed a decrease in the number of T lymphocytes and in the number of T helper cells, while the percentage of T suppressor cells and B cells was the same in both treated and untreated groups. It was also shown that the HLA-DR antigen expression on the epithelial cells was eliminated in the CyA treated patients. These findings suggest that the HLA-DR antigen expression on the epithelial cells is the result rather than the triggering factor of this T cell mediated process and is probably related to decreased lymphokine production by activated T lymphocytes.
2546568 Detection of clonally expanded salivary gland lymphocytes in Sjögren's syndrome. 1989 Jul Recurrent swelling of the parotid and submandibular salivary glands occurs in some patients with Sjögren's syndrome (SS). Using Southern blot methods, we analyzed immunoglobulin and T cell antigen receptor (TCAR) gene rearrangements in DNA obtained from salivary gland lymphocytes of 9 SS patients. Based on histologic appearance of the biopsy specimens, these patients were diagnosed as having myoepithelial sialadenitis. We found oligoclonal rearrangements of the kappa gene (4 patients) and lambda gene (1 patient), and oligoclonal rearrangement of the TCAR beta chain in 2 additional SS patients. Patients with Ig gene rearrangements did not show rearrangements of their TCAR gene, nor did patients with TCAR rearrangements exhibit Ig rearrangements. The observed oligoclonal rearrangements probably derive from 5-10% of the salivary gland B cells or T cells. Three of these SS patients developed non-Hodgkin's lymphoma 2-8 years after the initial biopsy. Our results suggest that minor populations of B cells or T cells may clonally expand in the salivary gland tissues of SS patients with pseudolymphoma, and that such lymphocyte expansions may be controlled by the endogenous immune response and/or medications. However, continued lymphoproliferation in these salivary gland tissues may eventually lead to emergence of a neoplastic clone that escapes immunologic control and develops into a non-Hodgkin's lymphoma as a result of a multistep process.
1882799 Duodenal ulcer and Sjogren's syndrome in patients with primary biliary cirrhosis: a casual 1991 Sep Peptic ulcer has been reported in patients with primary biliary cirrhosis (PBC), but its frequency and pathogenesis are still poorly defined. We have analyzed the occurrence of duodenal ulcer in 37 female patients affected by PBC and in 35 with chronic liver disease of various etiologies. An active ulcer was found in seven patients with PBC and in one with chronic autoimmune hepatitis. The presence of an exocrine gland defect, as indicated by clinical signs of Sjogren's syndrome (SS), was found in six patients with PBC and duodenal ulcer (85%), but in only eight (26.6%) of those without ulcer (p less than 0.02). Therefore, in our patients, duodenal ulcer occurs more often in PBC than in other types of chronic liver disease. The association of SS with PBC, significantly higher in patients with than without ulcer, supports the hypothesis that the underlying exocrine gland defect is involved in the development of duodenal ulcer.
1905419 [Detection of anti-Ro(SS-A) by using immunoimprint. Comparison with double diffusion in ag 1991 Mar Among soluble anti-nuclear antigen auto-antibodies, anti-Ro(SS-A) were, for unknown reasons, difficult to detect by immunoimprint (II). The aim of this study was the development of a method of preparation of Ro(SS-A) antigen enabling greater sensitivity in the detection by II of the corresponding antibodies, then application of this method in a series of 63 cases of primary Sjögren's syndrome in order to study the clinical significance of anti-Ro(SS-A) antibodies. Ro(SS-A) antigen was prepared from human spleen and partially purified on DE-52 resin. The protein extract prepared reacted in II with reference anti-Ro(SS-A) and anti-La(SS-B) antibodies. The series of 63 cases of primary Sjögren's syndrome (57 F, 6M) included 50 of extraglandular primary Sjögren's syndrome and 13 of isolated glandular primary Sjögren's syndrome. Twenty two cases of primary Sjögren's syndrome had anti-Ro(SS-A) (35%). Twenty patients had anti-Ro(SS-A) by II (32%) and 17 by double diffusion in agar (27%) (p = NS). Among 7 discordant sera, 2 were not detected by II. The incidence of anti-Ro did not differ statistically between the extraglandular (40%) and glandular (15%) primary Sjögren's syndrome groups. Patients were divided into two groups according to whether they did (n = 22) or did not (n = 41) produce anti-Ro(SS-A).(ABSTRACT TRUNCATED AT 250 WORDS)
2736831 Relations of the histopathology of sublingual and labial salivary glands to the clinical p 1989 Mar This study was designed to further analyse the histopathologic appearance of the sublingual and labial salivary gland biopsies performed in 50 patients suspected of having Sjögren's syndrome (SS). Three kinds of lesions were evaluated: occurrence of foci (an aggregate of more than 50 lymphocytes), lymphocytic infiltration of the duct walls, and the remaining abnormalities (such as scattered lymphocyte infiltrates, interstitial sclerosis, dilatation and regression of ducts and acini). The occurrence of foci correlates well with SS and even better when there is a lymphocytic infiltration of the ducts within the foci. The remaining changes were not specific for SS.
3497426 [Comparative profile of antinuclear antibodies in Gougerot-Sjögren syndrome with and with 1986 Nov Eleven patients with an isolated Gougerot-Sjögren syndrome and a diffuse interstitial fibrosis were compared with twenty patients with an isolated Gougerot-Sjögen without pulmonary involvement. Patients with pulmonary fibrosis are younger and the evolution of their dry syndrome is shorter (p less than 0.05) than in patients without fibrosis. The frequency of extra-articular clinical manifestations (except for the lung) is identical in both groups. Antinuclear antibodies are present in 100 p. cent of patients with pulmonary fibrosis. Specific antibodies of soluble nuclear antigens are detected in 64 p. cent of them. This frequency is 55 p. cent in the group without pulmonary fibrosis. The specificities of these antibodies are anti-U1-RNP (3 cases), anti-SS-B (La) (3 cases), anti-SS-A (Ro) (2 cases), non identified (1 case). There was no serum containing antibodies Jo1 or anti-Sm. This immunological profile is identical to the profile found in isolated Gougerot-Sjögren syndromes without pulmonary fibrosis. The search for specific antibodies of soluble nuclear antigens permits to differentiate pulmonary fibrosis secondary to an isolated Gougerot-Sjögren syndrome, from primary diffuse interstitial fibrosis and fibrosis associated to a polymyositis.