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ID PMID Title PublicationDate abstract
2832118 Paraneoplastic Sjögren's syndrome. 1987 Sep A patient with primary Sjögren's syndrome preceding an oat cell carcinoma of the lung, is presented. Arguments to support a possible relationship between these two disorders are discussed.
3473639 Treatment of xerostomia in patients with primary Sjögren's syndrome with sulfarlem. 1986 Stimulated by a recent report on the favorable effect of Anetholtritione (Sulfarlem S 25) on symptoms and salivary flow rate in patients with Sjögren's Syndrome (SS), we examined the effect of Sulfarlem in an open study on 16 patients characterized by severe xerostomia. Fourteen had primary SS and two xerostomia only. The patients were examined by whole resting saliva secretion rate measurement (SR) once a week during the study period of 7 weeks. At the same time the patients assessed their symptoms of xerostomia on a 1-10 visual analogue scale. Following the third examination (2 weeks), Sulfarlem was given p.o. 25 mg X 3 daily for 3 weeks, after which the patients were examined for another 2 weeks. The average SR before treatment was 0.07 ml/15 min. Two patients had increased secretion rates, but only one of these described improvement in symptoms. Two had improvement in symptoms and 12 had no positive subjective or objective effect from treatment. Side effects were abdominal discomfort and flatulence, seen in 7 patients (44%). One of these patients in addition had diarrhea and nausea. The side effects were persistent during treatment only. The medication was terminated following one week of treatment in two patients. It is concluded that Sulfarlem in a daily dose of 75 mg have no marked effect on salivation in patients with primary SS and severe xerostomia. Gastrointestinal side effects may occur.
3473626 Kidney involvement in primary Sjögren's syndrome. 1986 Kidney involvement is well recognized extraglandular manifestation of primary Sjögren's syndrome. The most common histopathological lesion is an interstitial lymphocytic infiltrate with tubular atrophy and fibrosis. In addition, immune complex associated glomerulonephritis has been reported in sporadic cases with primary Sjögren's syndrome. Here we present our experience on kidney involvement in 36 Greek patients with primary Sjögren's syndrome. The following routine renal laboratory tests were performed in all patients: serum creatinine, creatinine clearance, 24 hour urine protein excretion, urinalysis and plain film of the abdomen. These tests revealed two cases with nephrolithiasis and two with severe proteinuria (24 hour urine protein greater than 3.5 g). Kidney histology in the patients with proteinuria was compatible with membranous and membranoproliferative glomerulonephritis respectively. In fifteen randomly selected patients urinary acidification was studied before and after acute acid loading test (oral administration of NH4Cl). Renal tubular acidosis was found in five patients (one with complete and four with incomplete type). Seven patients voluntarily accepted renal biopsy; five of those had mild form of interstitial nephritis. Two of these patients had renal tubular acidosis. In conclusion, renal involvement (clinical and subclinical) is commonly found in primary Sjögren's syndrome patients.
3296146 Characterization of intraepidermal IgG deposits in patients with primary Sjögren's syndro 1986 In order to characterize the pathomechanisms behind intraepidermal in vivo deposits of IgG, which are found in 68% of patients with primary Sjögren's syndrome (primary SS), skin biopsies and serum from patients with epidermal IgG deposits were examined and compared to normal controls. Double-labelling experiments on skin biopsies, from 5 patients and 5 normal controls, showed that IgG deposits were predominantly located to surface membranes of OKT6 positive Langerhans cells. Only IgG1 and IgG3 were found deposited. Neither IgG2, IgG4, IgM, IgA, IgE, IgD, C1q, C3c, fibrinogen, albumin, beta-2 microglobulin nor C-reactive protein were found deposited in the epidermis of patients. Sera from 6 other patients with primary SS were examined for in vitro and in vivo binding of IgG to normal human epidermis. Using the athymic nude mouse/human skin model we were able to show that serum IgG from patients can be experimentally deposited in vivo in human skin transplants, but in vitro binding could not be demonstrated. The Fc-fragments of epidermal IgG were accessible to binding of anti-Fc-fragment antibodies and protein-A. We suggest that IgG-containing immune complexes constitute the intraepidermal IgG deposits seen in patients with primary SS, and that the binding possible is mediated by Fc-receptors of Langerhans cells and keratinocytes.
2450924 Nucleotide sequence of a DRw10 beta chain cDNA clone. Identity of the third D region with 1988 Apr 1 The nucleotide and inferred amino acid sequence of a DRw10 beta chain was obtained from cDNA clones isolated from a DR1, DRw10 heterozygous cell line. The sequence of this beta chain gene was distinctive, differing from those of all other defined DR types. The DRw10 beta chain gene was shown by transfection experiments to encode a polymorphic epitope recognized by mAb 109d6 that is also encoded by the DRw53 beta 2 chain gene. Comparison of the nucleotide sequence of both genes revealed that their third D regions (amino acids 67 to 73) were identical. This suggested first that the 109d6 epitope could be encoded by residues of this region, and second, that a putative gene conversion event transferred this sequence along with the information encoding the 109d6 epitope from a donor gene such as DRw53 beta 2. The sequence of the DRw10 beta chain gene was observed to be identical to that of clone pII beta 4 derived from the non-DR3 haplotype in the Raji cell line, which was also demonstrated to express the determinant recognized by antibody 109d6, suggesting that the typing of this cell line is HLA-DR3/DRw10. No evidence was found for the existence of a DR beta 2 chain gene product encoded by the DRw10 haplotype. The DRw10 haplotype was of particular interest because it was present along with a DR1 haplotype in the propositus who had rheumatoid arthritis, and was shared by the DR4-positive son of the propositus, who also had rheumatoid arthritis. This raised the possibility that the DRw10 haplotype, and most probably one or more specific conformations encoded by the DR beta chain, are involved in the definition of the disease susceptibility phenotype.
2696803 [A case of multiple cavities on chest film with high titer of serum cryptococcal antigen]. 1989 Nov A 26-year-old man admitted to Nishinomiya Municipal Hospital for further evaluation for abnormal shadows on the chest film in a mass examination. He had no subjective complaints; for example, cough, sputum or dyspnea. His past history and physical examinations yielded no significant findings. The chest film revealed the multiple cavities accompanied with a little infiltration throughout several lobes bilaterally. The inflammatory reactions; such as, CRP and ESR, were all intact, but the titer of the serum cryptococcal antigen was high. He was not immunocompromised. Although bronchoscopy and transbronchial lung biopsy were performed twice during admission, they revealed no pathogenic findings both bacteriologically and histologically. The elevation of the serum cryptococcal antigen titer suggested that this disease is the primary pulmonary cryptococcosis. The titer decreased with spontaneous disappearance of the abnormal shadows on the chest film. This test has a sensitivity of 90% for cryptococcal infection, but in rheumatoid arthritis, a small percentage of false positive results have been reported. Therefore, if the rheumatoid factor is eliminated, the test is more specific for cryptococcal infection. On the other hand, some authors have reported the false positive results in Trichosporon beigelii infection by this method. But this disease is negligible for its frequency in clinical features in our country. We emphasized that this non-invasive test is more useful for the diagnosis of the primary pulmonary cryptococcosis.
1798223 Primary amyloidosis with dry eyes and dry mouth--a case report. 1991 Nov We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated Sjögren's syndrome. Lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted in the urine. At autopsy, amyloid deposition was identified histochemically in many organs, mainly on the vessel walls. Primary amyloidosis should be considered as a differential diagnosis of Sjögren's syndrome.
2032427 Ectopic splenomegaly in Felty's syndrome. 1991 Mar A 50-year-old woman with Felty's syndrome, who presented with "menopausal" symptoms, was found to have a large pelvic mass on physical exam. Computed tomography of the pelvis led to an incorrect diagnosis of malignancy, while radionuclide imaging using Tc-99m sulfur colloid confirmed the diagnosis of ectopic splenomegaly.
1679104 Periodontal status of patients with Sjögren's syndrome: a cross-sectional study. 1991 Jan Sjögren's Syndrome (SS) is a chronic inflammatory disease characterized by xerostomia. Although it is confirmed that a common observation in SS is increased susceptibility to caries, the degree of influence of SS on periodontal disease has not been well-described. The purpose of this study was to determine the periodontal status of 14 SS female subjects and 14 age-matched females, who did not exhibit SS (controls). The mean age of the SS and control subjects were 52.9 +/- 11.6 and 53.7 +/- 8.9, respectively. The plaque (PI), gingival (GI), bleeding (BI) and calculus (CI) indexes, as well as probing depth (PD) and attachment level (AL), were estimated on 7 index teeth. Data were analyzed by means of the Student t-test. The results indicated that there were no statistically significant differences between the SS and control groups: PI, PD and GI (p greater than 0.05); AL and CI (p greater than 0.1); and BI (p greater than 0.01). Thus, it may be concluded from this study that Sjögren's syndrome has no observable influence upon the indices measured in evaluating periodontal disease.
2590083 The rarity--an acceptable stimulant. 1989 Sep Patients who present with uncommon or unusual or, occasionally, rare complaints help keep doctors vigilant. Such patients can stir the brain to action and prompt stimulating discussions with colleagues.
2349433 A follow-up study of pulmonary function in patients with primary Sjögren's syndrome. 1990 Twenty-seven patients (25 women, 2 men) with primary Sjögren's syndrome, previously reported to have reduced pulmonary diffusing capacities were reexamined in a 7-year follow-up in order to evaluate longitudinal alterations in pulmonary function. Primary Sjögren's syndrome was diagnosed according to the Copenhagen criteria. The present examination revealed normal and unchanged values for vital capacity, forced expiratory volume in 1 s, maximal expiratory flow at 50% of expired vital capacity (MEF50), and diffusing capacity per liter alveolar volume. Total diffusing capacity (P less than 0.01) and MEF75 (P less than 0.05), were, however, significantly reduced compared with the predicted values, indicating pulmonary involvement primarily affecting the small airways. The longitudinal examination, furthermore, showed increasing values for total diffusing capacity (P less than 0.02), diffusing capacity per liter alveolar volume (P less than 0.001), and MEF75 (P less than 0.02), suggesting an improvement in lung status in the course of time. No correlation was found between MEF75 and diffusing capacities, nor between alterations in pulmonary function and complaints of dyspnoea, tiredness, cough, expectoration, tobacco smoking, or medical treatment with bromhexine, glucocorticosteroids, essential fatty acids, or nonsteroid antiinflammatory drugs.
2057729 [Action of non-steroidal anti-inflammatory agents on the immune system]. 1991 May It is widely admitted that the non steroidal anti-inflammatory drugs (NSAID) inhibits the synthesis of prostaglandins by blocking the membrane cyclo-oxygenase. The anti-inflammatory activity of these molecules is partly explained by the vaso-dilatational action of PG2 in particular. However this effect alone cannot account for all the properties of NSAID. The latter have an inhibitory action at the level of the various functions of neurophil leucocytes and to a lesser degree at the level of macrophages. For immune system itself, it seems the NSAID have a rather immunostimulant effect due to a major action on T lymphocytes. In the course of rheumatoid arthritis (RA), the NSAID are unable to modify the ratio CD4/CD8. Yet they may decrease the production of the rheumatoid factor (RF). This ability is related to a loss of the normal suppressive T cells inhibition exerted by PG. Besides the NSAID seem unable to modify the natural killer function (NK). Finally, the impact on the synthesis of interleukins (IL) notably 1 and 2 does not seem clear. Indeed several research papers give us contradictory results between animals and men and between physiological or pathological situations.
1656245 The interaction of cryoimmunoglobulins with a model surface. 1991 Sep Cryoimmunoglobulins are associated with numerous clinical problems ranging from collagen vascular disorders (rheumatoid arthritis and systemic lupus erythematosus) to infectious processes including HIV infection. The precise role of cryoglobulins in the pathophysiology of these disorders remains unresolved. Although cold insolubility may account for some of the observed processes, it cannot explain the entire array of findings in cryoglobulinemia. An alternative hypothesis suggests that the subtle differences responsible for cold precipitation of these proteins renders them intrinsically more sticky, resulting in deposition of cryoimmunoglobulins on vascular surfaces. We have explored this hypothesis by characterizing the binding of monoclonal cold soluble and cryoimmunoglobulins to silica beads as a model biological surface. It is found that monoclonal, type I, IgM and IgG cryoglobulins have only a slight tendency to bind to a greater extent to this surface than cold soluble immunoglobulins. Physical studies utilizing front surface fluorescence measurements and differential scanning calorimetry show surface interaction leads to partial thermal destabilization of the proteins. To a limited extent, this destabilization is more pronounced with the cryoglobulins compared to cold-soluble control homologues. Surface bound IgM cryoimmunoglobulin was also found to fix complement less efficiently than their cold soluble surface bound counterparts. These studies do not strongly support the hypothesis that pathological mechanisms of cryoimmunoglobulins primarily involve abnormal surface interactions, although surface effects could play a limited role in some situations.
2691147 A method for retracing the putative antigen/antibody ratio of immune complexes in biologic 1989 Sep It is well known that immune complex (ICs) diseases are caused by a number of factors which influence the localization, clearance and inflammatory potential of ICs. Several studies suggest that the Ag/Ab ratio is one of the most important of these. Previous studies have clarified that IC detection methods which differ, either in their recognition unit or in the phase used (solid or liquid), show a very poor correlation with each other. This study was developed in order to verify the hypothesis that different methods recognise different kinds of ICs on the basis of their Ag/Ab ratio. We used 3 homogeneous EIAs employing a probe complex enzyme--anti enzyme which competes with circulating ICs for the recognition unit (bovine conglutinin, C1q or monoclonal rheumatoid factor) to detect 10 unrelated in vitro-made ICs at different relative Ag/Ab concentrations (from 8 x Ag excess to 8 x Ab excess). We demonstrated that the 3 recognition units recognised the ICs principally on the basis of their Ag/Ab ratio. These results were then used to set up a mathematical model capable of retracing the Ag/Ab ratio of the ICs present in unknown samples. This was employed to test a panel of sera from patients with systemic lupus erythematosus, essential mixed cryoglobulinemia and rheumatoid arthritis; we obtained very suggestive results but they require further prospective studies to understand the full significance of this parameter.
1879893 Occurrence of systemic autoimmune disorders & autoantibodies in house-hold contacts of pat 1991 Apr Household contacts of 22 randomly selected patients with systemic lupus erythematosus (SLE) were screened for autoimmune diseases and autoantibodies. Thirty nine consanguineous and 17 nonconsanguineous household contacts were studied. In the first group symptomatic SLE was seen in 5 per cent, rheumatoid arthritis in 2.5 per cent, antinuclear antibody (ANA) in 10 per cent and rheumatoid factor (RF) in 5 per cent while anti-dsDNA, anti-nRNP and anti-Sm antibodies were not detected in any individual. The second group showed total absence of any marker of autoimmunity. In normal controls only RF was detected in 3 per cent. The occurrence of markers of autoimmunity only in consanguineous household contacts of patients with SLE further confirms the recognised role of genetic factors in the etiology of SLE.
1700939 Sjögren's syndrome: an oligo-monoclonal B cell process. 1990 Jul Primary Sjögren's syndrome (1 degrees SS) has been considered as a privileged model for the study of autoimmunity and B-cell neoplasia. Previous and recently accumulated information have reinforced this view. The given higher incidence of non-Hodgkin's lymphoma (NHL) in 1 degree SS patients, the presence of circulating monoclonal immunoglobulins, the detection of uniform immunoglobulin gene rearrangements and monoclonal B-cell expansions in the lymphocytic infiltrates of salivary gland, the increased levels of circulating CD5 positive B-cells and the association of these cells with the presence of monoclonal immunoglobulins from 1 degree SS, and finally the finding of shared cross reactive idiotypes on monoclonal immunoglobulins from 1 degree SS and B-cell malignancies, all provide evidence of common pathogenetic links between benign and malignant lymphoproliferation.
2169602 Amplification of Epstein-Barr virus genomic sequences in blood cells, lacrimal glands, and 1990 Aug Based on observations of primary Sjögren's syndrome (SS) following acute Epstein-Barr virus (EBV) infection, the authors hypothesized that EBV may play a role in the pathogenesis of SS. This hypothesis was tested by evaluating ten peripheral blood mononuclear (PBMN) cell specimens, ten lacrimal gland biopsies, and five tear specimens from 15 EBV-seropositive primary SS patients for EBV genomic sequences using polymerase chain reaction (PCR). Epstein-Barr virus DNA sequences were detected in 50% of SS PBMN cell specimens and 80% of SS lacrimal gland and tear specimens. In six SS patients, specimens were obtained from two or more sites (i.e., PBMN cell and lacrimal gland and/or tears), and EBV genomic sequences were amplified in the PBMN cells and the lacrimal gland or tears in three of these subjects. The authors previously detected EBV genomes in 32% (11/34) of normal human lacrimal glands from EBV-seropositive donors using PCR and concluded that the normal human lacrimal gland may be a site of EBV persistence; however, they were unable to amplify EBV sequences in DNA from PBMN cells or tear specimens from normal donors. Amplification of EBV DNA in PBMN cells, lacrimal glands, and tears of primary SS patients at a greater frequency (P less than 0.01) than normal controls suggests that EBV may be a risk factor in the pathogenesis of SS.
2429977 Significance of the Ro antigen system. 1986 Sep Knowledge about antibodies to the Ro/SSA and La/SSB antigens has expanded greatly. Recognition of these antibodies was probably achieved 25 years ago but their macromolecular structure, clinical associations, and genetic relationships have come to light only in the past 7 years. It seems clear that these antibodies have a special place in the nosology of SLE and SS and that in certain instances (e.g., neonatal LE) the antibodies play a direct pathogenic role, while in other circumstances (e.g., vasculitis, nephritis, SS) tissue damage might result from immune complex deposition on vascular structures. Certainly, the latter problems will be active areas of investigation in the coming years. If the pace of recent progress continues, many of the questions raised in this review should soon have clear answers.
2551309 Possible involvement of Epstein-Barr virus (EBV) in polyclonal B-cell activation in Sjögr 1989 Aug The following results suggest that EBV might be involved in the mechanism of polyclonal B-cell activation of Sjögren's syndrome (SS). (1) The levels of serum anti-VCA antibodies of both IgG and IgM class were significantly elevated in SS. (2) Excretion of EBV from the oropharynx was frequently observed in SS. (3) Spontaneously transformed B-cell lines producing a large amount of transforming EBV were established preferentially from SS. (4) An EBV-specific regulatory mechanism was impaired in SS.
3492207 In vitro synthesis of IgM rheumatoid factor in response to Staphylococcus aureus, by lymph 1986 Dec Peripheral blood mononuclear cells from 20 healthy adults were tested in vitro for the production of IgM rheumatoid factor (RF) in response to Staphylococcus aureus Cowan I (SAC) or pokeweed mitogen. Fifteen of the 20 normal subjects produced greater than or equal to 4 ng/ml IgM-RF (mean +/- SD 46 +/- 55 ng/ml) in response to SAC, compared with only 2 of 20 who produced greater than or equal to 4 ng/ml IgM-RF (mean +/- SD 2 +/- 4 ng/ml) in response to pokeweed mitogen (P = 0.0001). Separation and reconstitution of autologous T and B cell-enriched fractions, with and without prior T cell irradiation, provided evidence for a radiosensitive T helper/inducer cell involved in the IgM-RF response to SAC in 70% of the normal subjects studied. SAC appears to be a potent stimulus of IgM-RF production, with a cellular mechanism distinct from that of other in vitro systems.