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ID PMID Title PublicationDate abstract
2291072 [Gougerot-Sjögren syndrome associated with dysthyroidism]. 1990 Nov The occurrence of Gougerot-Sjögren syndrome in a context of dysthyroidism is not a chance happening, as is reflected by the 37% rate of incidence. Routine labial biopsy, carried out in 59 cases of primary hypothyroidism due to atrophic thyroiditis, 20 of Basedow's disease and 4 of Hashimoto thyroiditis identified Gougerot-Sjögren syndrome (3-4 Chisholm grade) in 3 of the 59 cases of hypothyroidism i.e. in 5 percent, and in one Basedow case out of 20, i.e. 5 percent. Using Kaplan's criteria, the incidence of Gougerot-Sjögren syndrome rises to 22 percent in hypothyroidism and 30 percent in Basedow's disease. Consequently, the incidence of Gougerot-Sjögren syndrome is higher in Basedow disease than in primary hypothyroidism. Gougerot-Sjögren syndrome is seen particularly in dysthyroidism involving manifest and all-round clinical signs. This association does not seem to result from one disease being caused by the other, but the outcome of the onset of entities to which a genetically determined context predisposes the subject, as is shown by the prevalence of HLA DRW3 and HLA B8 groupings in Gougerot-Sjögren syndrome, in primary hypothyroidism, in Basedow's disease and in Hashimoto's thyroiditis.
2399428 Localization of lysozyme mRNA in the labial salivary glands by in situ hybridization in Sj 1990 Aug In this study, lysozyme mRNA in labial salivary glands has been localized with in situ hybridization technique using 35S-labeled hen lysozyme cDNA (cDNALZM) as a hybridization probe in normals and in patients with Sjögren's syndrome, 35S-DNALZM:mRNA hybrids were detected only in acinar serous cells, although lysozyme was identified in ductal cells using immunohistochemical techniques. Our results suggest that the serous acinar cells are the only site of lysozyme synthesis in small salivary glands. The presence of lysozyme in ductal cells may be a result of reabsorption from the saliva or concentration from the blood or surrounding tissues.
2590099 Connective tissue disease mimicking multiple sclerosis. 1989 Oct Systemic lupus erythematosus, primary Sjögren's syndrome and systemic sclerosis may be associated with acute transverse myelitis and chronic relapsing neurological syndromes mimicking multiple sclerosis in the same individuals and/or their relatives. We now present three cases which suggest that there is a wide spectrum of connective tissue disorders mimicking multiple sclerosis and acute disseminated encephalomyelitis. These cases demonstrate that the diagnosis of multiple sclerosis should be kept under constant review by searching for the development of connective tissue disorders in the patients or their relatives.
2529304 The uses of intravenous immune globulin in collagen vascular disorders. 1989 Oct Studies in our laboratory demonstrated that the long-term administration of intravenous immune serum globulin in patients with common variable hypogammaglobulinemia produced increased suppressor activity, which resulted in diminished in vitro B cell immunoglobulin synthesis. These studies suggested that intravenous immune globulin therapy might be a useful modality in altering immunoregulation in patients with collagen vascular autoimmune disease. In the present preliminary study, two groups of patients with collagen vascular disease were chosen: systemic lupus erythematosus and primary Sjögren's syndrome. Patients with systemic lupus erythematosus with mild clinical disease were chosen to minimize the risk of adverse effects on the disease process, particularly specific organ involvement (e.g., lupus nephritis). Each patient was used as his or her own clinical and laboratory control. Patients received 300 mg/kg of intravenous immune globulin every 4 weeks. As a whole the patient group did not experience any adverse effects from the intravenous immune globulin therapy. No clinical or laboratory changes were observed in one patient with systemic lupus erythematosus and one patient with Sjögren's syndrome. In the other patient with Sjögren's syndrome, there were subjective changes of improved well-being and increased energy levels without any objective changes in the sicca syndrome. There was a slight steroid-sparing effect (10 to 3 mg/day) but no effects on the sedimentation rate, the antinuclear antibody, or rheumatoid factor serologic studies. In a patient with steroid-dependent systemic lupus erythematosus, there was marked clinical improvement of the cutaneous vasculitis after the third infusion, with reduction in her oral steroid requirements from 25 to 7.5 mg/day.(ABSTRACT TRUNCATED AT 250 WORDS)
3050578 [Involvement of the renal interstitium in systemic diseases (I)]. 1988 Interstitial lesions which are frequent in systemic diseases such as systemic lupus erythematosus, Sjögren's syndrome, sarcoidosis and Dobrin's syndrome (acute interstitial nephritis with uveitis) are described as well as their clinical expression. The immune reactions which are recognized to be responsible for these lesions are discussed in respect to each of these diseases.
3579959 Autoantibodies against RNA polymerase I in scleroderma and Sjögren's syndrome sera. 1987 May 14 Anti-RNA polymerase I antibodies were detected by radioimmunoassay in the sera of 8 out of 9 Sjögren's syndrome patients, 11 out of 19 individuals with scleroderma, and 19 out of 19 systemic lupus erythematosus patients. The results of the radioimmunoassay were confirmed by demonstrating the ability of the patients' IgG to inhibit RNA polymerase I activity in vitro. Sera from four patients in each category were also tested for reaction with individual subunits of RNA polymerase I. All 4 SLE sera, 3 out of 4 scleroderma sera and 1 out of 4 Sjögren's syndrome sera contained antibodies against the 65 kDa (S3) subunit of RNA polymerase I in addition to antibodies against one other subunit. Sera from the remaining scleroderma and Sjögren's syndrome patients tested in this assay contained only anti-S3 antibodies. These results demonstrate that anti-RNA polymerase I antibodies are characteristic of a variety of rheumatic autoimmune sera.
3527087 HLA-DR expression by labial minor salivary gland tissues in Sjögren's syndrome. 1986 Aug Minor salivary gland biopsy specimens from patients with Sjögren's syndrome (primary and secondary) and from normal controls were examined with the four step biotin-avidin-immunoperoxidase assay. The composition of the infiltrating cells was similar in patients with both primary and secondary Sjögren's syndrome, consisting primarily of T lymphocytes with predominance of T helper/inducer cells. B lymphocytes (Leu-14) were approximately 20-35% of the infiltrating lymphocytes, while only a few OKM1 (monocytes/macrophages) cells and Leu-7+ (natural killer; NK) cells were observed. The majority of infiltrating lymphocytes expressed HLA-DR antigens. In the biopsy specimens of the controls there were no infiltrates; the scattered lymphocytes, however, were also predominantly T lymphocytes. Finally, the glandular epithelial cells (ducts and acini) were inappropriately expressing HLA-DR antigens, in contrast with controls where minimal HLA-DR expression was found.
2898315 Restriction fragment length polymorphism in Felty's syndrome. 1986 Jun Southern blot analysis with DR beta and DQ beta cDNA probes was used to compare genomic DNA from Felty's syndrome patients with HLA-DR-matched normal controls. We describe two restriction fragment length polymorphisms putatively associated with Felty's syndrome.
1751307 The molecular genetics of systemic lupus erythematosus and Sjögren's syndrome. 1991 Oct With recent advances in understanding of the organization, complexity, and function of the major histocompatibility complex and other genes involved in the immune response, a rational approach to interpreting associations of systemic lupus erythematosus and Sjögren's syndrome with these genes is now possible. For example, both systemic lupus erythematosus and Sjögren's syndrome appear to be heterogeneous syndromes comprised of different major histocompatibility complex-mediated (and perhaps T-cell receptor-and immunoglobulin genes-mediated) autoantibody responses. Moreover, HLA class II associations with systemic lupus erythematosus- and Sjögren's syndrome-linked autoantibody responses seem to be better explained by specific amino acid residues or epitopes on these class II heterodimers, residues or epitopes putatively involved in the binding and presentation of processed antigen to the T-cell receptor that can be shared by different class II alleles. These basic themes underlie this review of recent work in the immunogenetics of these autoimmune diseases.
1648423 [Possible etiological relations between Sjogren's syndrome and Epstein-Barr virus]. 1991 Mar Tissue biopsies from patients with Sjogren's syndrome (SS) and controls were detected by a monoclonal antibody (McAb) of gene engineering against Epstein-Barr virus (EBV) encoded early antigen (EA p138) and a 32p-labelled EBV DNA probe, and also B95-8 and K4 cells as antigens to detect reacting antibodies to EBV in patients' sera. As a result, cytoplasmic fluorescence staining of epithelial cells with the McAb as described above was noted in 15/33 labial and 7/7 renal biopsies from primary SS. The same McAb did not react with biopsies from secondary SS, non-SS connective tissue diseases, benign tumors and normal persons, nor with the other tissues detected from primary SS. By using McAbs against the EBV EA p54 and nuclear antigen (EBNA-1) and by using immunoblotting method, it was found that the reacting antigens in labial biopsies of primary SS had both a M. W. of 54,000 and a M. W. of 65,000, similar to the EA-D and EBNA-1 antigens found in lymphoblastoid cells lytically infected with EBV. Seven out of 21 labial and one out of two renal samples from primary SS patients contained EBV DNA detectable by dot blot hybridization with EBV Bam W probe. Serum levels of both antiviral capsid antigen antibodies and antinuclear antibodies were elevated in patients with primary SS. Our results demonstrated an elevated content of EBV in labial gland and kidney of patients with primary SS, where EBV has been of a lytic fate leading to active replication. It is concluded that EBV may play an important role in the pathogenesis of primary SS.
2196506 Oral mucosal status and major salivary gland function. 1990 Jul Normal salivary function is considered to be critical for the maintenance of healthy oral mucosa. However, few studies have examined mucosal changes in patients with objectively documented salivary gland performance. In the present report, the mucosal status of 298 subjects being evaluated in a dry mouth clinic was assessed. A complete oral examination was performed and unstimulated and stimulated salivary samples were collected separately from the parotid and submandibular/sublingual glands. Data were analyzed according to diagnosis and salivary output after the assignment of an oral mucosal rating to each subject. In general, the mucosal surfaces were well preserved and infections were not seen. Patients evaluated for Sjögren's syndrome and radiation-induced xerostomia had the lowest salivary gland performance but displayed a mucosal status similar to denture-wearing healthy subjects or patients with normal salivary flow who had idiopathic xerostomia. However, those patients with a total lack of salivary flow rarely had normal-appearing oral mucosa. These results confirm a role for saliva in oral mucosal preservation and also suggest that other factors may act to maintain oral mucosal integrity.
2909222 Computed tomography of salivary glands in Sjögren's syndrome. 1989 Jan Three cases of Sjögren's syndrome, one with uncomplicated disease, and the other two complicated by pseudolymphoma and malignant lymphoma, respectively, are presented. Computed tomography can provide information useful in the diagnosis, detection of complications, and, therefore, management of Sjögren's syndrome.
3266361 Circulating IgM rheumatoid factors in patients with primary Sjögren's syndrome. 1988 The importance of circulating rheumatoid factors (RF) in primary Sjögren's syndrome (primary SS) was evaluated retrospectively by examining medical case records of 80 consecutive patients. Increased levels of IgM RF, determined by the Waaler test, the latex fixation test and/or the ELISA test, were found in 47 patients (59%). Follow-up examination of the 41 patients in whom more than one (mean 4.9 (2-12)) RF determination over at least a two-year period (mean 5.6 (2-13) was present, showed that 12/41 patients (29%) were permanently RF-negative, 7/41 (17%) exhibited both positive and negative RF values and 22/41 (54%) were permanently RF positive. Variations in IgM RF levels were unrelated to disease duration. Except for involvement of joints, extraglandular manifestations were more common in patients with increased levels of RF. This finding, however, was only significant within the group of more rarely occurring extraglandular manifestations (serositis, interstitial nephritis, cutaneous vasculitis, lymphoproliferative disorders and intermittent fever) (p less than 0.01). IgM RF levels were likewise positively correlated (p less than 0.001) to positivity of IgG antinuclear antibodies as well as to the plasma concentrations of immunoglobulins.
3313514 [Sialographic and sonographic diagnosis of salivary gland diseases]. 1987 Jun The diagnostic value of sialography and ultrasonography was studied retrospectively in 130 patients with diseases of the salivary gland. In the diagnosis of tumours (sensitivity: 97.5% vs 52.5%), acute inflammatory diseases (sensitivity: 70% vs 40%) and abscesses (sensitivity: 100% vs 25%) ultrasound was more accurate than sialography. Even in the diagnosis of calculi, ultrasound gave good results (sensitivity: 71.4%) and should be performed before the more invasive sialography technique. In chronic inflammatory diseases, sialography continues to be the technique of choice.
2943291 Complement C3b receptors on erythrocytes, circulating immune complexes, and complement C3 1986 Jul Twenty-one patients with primary Sjögren's syndrome (SS) and 22 age- and sex-matched healthy controls were examined for levels of C3b receptors (CR1) on erythrocytes (by an enzyme-linked immunosorbent assay), levels of circulating immune complexes (IC) (by a polyethylene glycol precipitation complement consumption method), and C3 split products, C3d and C3c (by intermediate gel rocket immunoelectrophoresis). CR1 varied within a wide range (20-124%), and a preponderance of low numbers was found among patients with primary SS (P less than 0.05). The levels of circulating IC and C3d were elevated in primary SS patients (P less than 0.01) and were mutually correlated (P less than 0.01; tau = 0.5), but levels of circulating IC and C3d did not correlate with CR1 values. C3c concentrations were the same in patients and controls. It is possible that the CR1 level serves as a predictor for the development of IC-induced manifestations, and the C3d concentration serves as an objective parameter for disease activity in primary SS.
3485480 Interleukin 2 augmentation of the defective natural killer cell activity in patients with 1986 Jan Natural Killer (NK) cell activity against K562 target cells was measured in 21 female patients with primary Sjögren's syndrome (primary SS) and in 20 female normal controls matched for age. The in vitro effect of alpha-interferon (IF) and interleukin 2 (IL-2) on NK cell activity was examined and the percentage of large granular lymphocytes (LGL) in blood was measured. Median baseline NK cell activity in primary SS was 15.4% versus 24.4% in the controls (P less than 0.05). Median IF-enhanced NK cell activity in the SS group was 35.5% versus 49.6% in the controls (P less than 0.02). IL-2-enhanced NK cell activity was 35.5% versus 37.6% in the controls (n.s.) The proportion of LGL did not differ in the two groups. Median LGL/lymphocytes was 4.0% in the primary SS patients versus 4.5% in the controls (n.s.). We conclude that the defective NK cell activity in patients with primary SS is functional, as the number of LGL is normal. Further the NK cell activity off SS was restored by IL-2.
2536549 Computed tomography and ultrasonography in parotid tumors. 1989 Jan To determine the most appropriate radiographic approach to parotid tumors, computed tomography (CT) was performed in 65 patients with 33 benign tumors, 19 malignant tumors, 3 abscesses and 10 other lesions with a clinical appearance of tumor. In 22 of the 65 cases CT-sialography and in 26 ultrasonography (US) were also performed. The mass was well recognized on CT without sialography in all patients, and the lesion was also well evaluated in all those examined with US. At CT-sialography the tumor was well shown, but this method made it difficult to differentiate a benign from a malignant lesion, because the character of the delineation and the border of the tumor were difficult to evaluate. It is concluded that US and CT without sialography are satisfactory for evaluating the parotid tumor, and the indication for CT-sialography is limited to those cases in which the tumor is demonstrated on US, but is unclear on conventional CT.
3473649 Focus score in the diagnosis of Sjögren's syndrome. 1986 According to current agreements, the verification of SS should consider all three components of diagnostic relevance: focal sialadenitis, keratoconjunctivitis sicca and an associated disease. Focus score (the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4 mm2 glandular section) of greater than 1 is probably the single most important test result in the diagnosis of the oral component in SS. Without such quantitation mild, nonspecific inflammation is often falsely classified as focal sialadenitis suggesting an underlying SS. This was confirmed by a retrospective study of a slide library, which showed that most cases descriptively diagnosed as focal sialadenitis actually had focus scores of less than 1. Furthermore, conditions other than SS can result in focus scores of greater than 1. Firstly, in 8/17 patients, sialolithiasis of the submandibular glands was associated with focus scores of greater than 1, usually with little or no histological signs of obstruction and/or infection. Secondly, focus score values of greater than 1 were found in the labial salivary glands in 6/40 coroner's autopsies although the subjects had no clinical history or findings suggesting SS; simultaneous specific involvement of both salivary and lacrimal glands was, however, observed in 0/40 of the subjects. Based on our observations we suggest that a postmortem diagnosis of SS should be made on the demonstration of focus score of greater than 1 in both the labial salivary glands and the lacrimal glands.
2084862 [Breast cancer and B cell malignant lymphoma associated with Sjögren'ssyndrome--a case re 1990 Oct It is well known that some patients with Sjögren's syndrome develop lymphoproliferative disorders. Approximately 60 cases of Sjögren's syndrome associated with malignant lymphoma, predominantly of B cell origin, have been reported in Japan. On the other hand, fourteen cases of non lymphoid malignant neoplasm, including six of thyroid cancer and four of gastric cancer, have been recently presented in Japanese literature. However, cases of double malignant neoplasms in Sjögren's syndrome are very rare. We reported a case of Sjögren's syndrome associated with breast cancer and B cell malignant lymphoma in the paper. A 53-year-old female was seen with eye pain, xerostomia, finger pain and right parotid swelling in January, 1985. Keratoconjunctivitis sicca was found. She had had eye pain and Raynaud's symptom during the past seven years. Sialectasia of grade III by Rubin and Holtz was seen in sialography. Schirmer's test was positive. Pathological finding of sialadenitis in minor salivary gland confirmed diagnosis of Sjögren's syndrome. Laboratory findings showed leukocytopenia and polyclonal hypergammaglobulinemia. Rheumatoid factor and antinuclear antibodies including SS-A antibodies were positive. Tuberculin test was negative. Ratio of CD 4 per CD 8 T lymphocytes was increased in peripheral blood. She had history of breast cancer. Radical mastectomy, followed by irradiation was performed in 1980. Arthralgia and parotid enlargement were transiently diminished by administration of small amount of prednisolone. She was readmitted because of progressive bilateral parotid swelling in April 1988. Pathological finding of the parotid was consistent with malignant lymphoma of diffuse, small cell type (lymphoplasmacytic). Immunoperoxidase staining revealed positive cytoplasmic IgM, k in lymphoma cells.(ABSTRACT TRUNCATED AT 250 WORDS)
3071138 Membranoproliferative glomerulonephritis in a patient with primary Sjögren's syndrome. Re 1988 Glomerulonephritis complicating primary Sjögren's syndrome is extremely rare, with only 3 cases of membranoproliferative glomerulonephritis reported in the literature. We report on a 55-year-old woman with a long-standing history of dryness of the mouth and eyes who was found to have nephrotic syndrome. Extensive investigations indicated primary Sjögren's syndrome. Kidney biopsy revealed a membranoproliferative glomerulonephritis. Treatment with prednisone and cyclophosphamide resulted in complete remission of nephrotic syndrome. The pathogenesis of glomerulonephritis appears to be due to deposition of circulating immune complexes.