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| ID | PMID | Title | PublicationDate | abstract |
|---|---|---|---|---|
| 1886672 | Inflammatory pseudotumor of the choroid plexus in Sjögren's disease. | 1991 Aug | We report an unusual case of inflammatory pseudotumor of the choroid plexus of the right lateral ventricle, manifesting as an intraventricular mass and causing unilateral hydrocephalus in a 48-year-old man who suffered from Sjögren's disease and subacute cutaneous lupus erythematosus. The lesion obliterated the normal choroidal architecture by a mixed chronic inflammatory process that was associated with reactive connective tissue changes. Immunohistochemical studies showed no light-chain restriction in the cells, and residual islands of transthyretin-positive epithelial cells were identified, implicating the choroid plexus origin of the mass. | |
| 2050903 | Clinical and histological analysis of labial lip biopsy in Sjögren syndrome. | 1991 Jan | Labial lip biopsy findings from 52 patients with Sjögren syndrome (SjS) and cutaneous manifestations were clinically analyzed. Findings of the labial lip biopsy with more than one focus (a cluster of at least 50 lymphocytes) were positive in 52% (28/52). Sixteen patients who presented with oral dryness had negative labial lip biopsy findings with less than one focus score and five patients without oral dryness had positive results. Cells infiltrating into the salivary gland were categorized as plasma cell rich (Group 1), lymphocyte rich (Group 3), intermediate (Group 2), or no gland in biopsied specimens (Group 4). Sicca complaint and the grade of lip biopsy score were significantly higher in Group 3 than in Group 1; no differences were observed in laboratory findings between Groups 1 and 3. The patterns of age distribution and salivary flow rate were also not statistically significant in these two groups. The patients with plasma cell rich infiltrate presented oral dryness, although their clinical and grade of lip biopsy scores were relatively low; most of these patients have secondary SjS. Some of the patients in Group 4 showed an advanced stage of the disease through their lip biopsy score was grade 1 with atrophic or no gland. These data suggest that labial lip biopsy is a useful screening method for the diagnosis of Sjögren syndrome and that clinical manifestations may differ in parallel with the type of infiltrating cell. | |
| 2261706 | Sequential salivary scintigraphy in Sjögren's syndrome: proposal for a new method of eval | 1990 Sep | Thirty-seven non-elderly primary Sjögren's syndrome (I SS) patients, and 27 controls complaining of xerostomia and/or recurrent or persistent swelling of at least one parotid or submandibular gland due to other selected disorders, were studied. A new scintigraphic score (scsc) is proposed for a standardised semiquantitative evaluation of salivary involvement by qualitative sequential salivary scintigraphy (SSS), and is compared with two other well-known methods generally used for this purpose. The scsc proved to be much more suitable for such an evaluation: the other criteria did not allow us either to classify or to score a great number of cases. Furthermore, the scsc allowed us to obtain better SSS sensitivity (89.2%) and specificity (96.3%) values for I.SS (33 of the 37 I SS patients and 26 of the 27 controls were correctly classified by discriminant analysis, for scsc values greater than 8). This was made possible by the different "weights" which we assigned to the various glandular and oral parameters in the scsc determination. | |
| 2358098 | [The role of drugs, irradiation, autoimmune diseases and age in the development of xerosto | 1990 May | The effects are described of xerostomia on the oral mucosa and dental structures. The various conditions which give rise to extreme dryness are listed; a rough estimate is made on the prevalence of these conditions. The contribution of drug induced xerostomia, of post irradiation mouth dryness and of autoimmune associated salivary disorders are dealt with. Reference is made to studies of xerostomia in various subpopulations. The prevalence of diseases known to be associated with mucosal dryness, and the yearly patterns of drug prescription in Ireland are used to justify an estimate of 2.1% to 3.3% of the population to be the incidence of xerostomia in the Republic of Ireland. | |
| 3513793 | Morphometry in the diagnosis of Sjögren's syndrome. | 1986 Mar | Sublabial salivary gland biopsies of 20 patients with Sjögren's syndrome and 58 controls were analyzed morphometrically to determine which histologic changes in the tissue are specific enough to justify a diagnosis of Sjögren's syndrome and which changes are due to physiologic aging. The acinar atrophy, fibrosis, ductal hyperplasia and ductal dilatation mentioned in the literature as features of Sjögren's syndrome are also observed in the tissue of aging individuals, and the lymphocytic focus score cited as the most important diagnostic parameter gives rise to about 9% of false-positive diagnoses. When using single quantitative histologic parameters, the volume percentages (Vol%) of lymphocytic foci, diffuse lymphoplasmacytic infiltrate (DLPI), acini and the inner diameter of intralobular ducts (ILD) were able to discern between the patients and the controls at a significant level, regardless of age, although considerable overlap was still present. This overlap could be reduced by consideration of at least two histologic parameters. The inhomogeneity within the tissue constituents was also used in discriminating between the patients and the control subjects. The best bivariate discriminating combination of histologic parameters was Vol% of lymphocytic foci and DLPI. Compared with qualitative subjective evaluation, this morphometric decision rule in the present material gave a 5 X reduction in the number of false-positive diagnoses of Sjögren's syndrome, with only 1 of the 58 control subjects erroneously classified as having the syndrome. We conclude that quantitative investigation of sublabial salivary gland tissue will improve the diagnostic criteria needed for the definition of Sjögren's syndrome. | |
| 2736304 | [Morphologic characteristics of the parotid glands in monkeys in the late period following | 1989 May | Three monkeys of "Macaca iris" species were examined twice: 3 months and 1 year after having mumps. Morphological data of both parotid glands biopsy are presented. Macrophage lymphocytes and plasmatic cells infiltration around the widened ducts as well as between the acini, growth of fibrosis and lipomatosis, and secretory stasis in glandular cells were observed long time after the disease. These changes are typical of Sjögren disease. This fact allows the authors to consider that virus affection may also be important in the aetiology of parotid glands autoimmune processes. | |
| 2716199 | [Development of acute myelocytic leukemia in the course of Sjögren's syndrome]. | 1989 Jan | A 53-year-old female, who had been having xerostomia and xerophthalmia for two years, was admitted in November 1984, because of subfever and malaise. Laboratory examination revealed pancytopenia with appearance of blasts, mild hypergammaglobulinemia, and elevated titers of ANA, anti SS-A and anti SS-B. Bone marrow aspirate showed increased myeloblasts. Schirmer's test and Rose Bengal's test were both positive, and sialography showed apple tree like appearance. Pathological findings of biopsied lip mucosa showed remarkable infiltration of lymphocytes. The patient was diagnosed acute myelocytic leukemia and Sjögren's syndrome. In spite of recovery of peripheral blood after combination chemotherapy (BHAC-DMP), a disturbance of consciousness appeared and she died of its progression. It was suspected clinically that she was suffered from progressive multifocal leukoencephalopathy (PML). It is sometimes reported that patients with Sjögren's syndrome develop malignant lymphoma, but it is very rare that Sjögren syndrome develop AML. | |
| 3235802 | Esophageal dysfunction in primary biliary cirrhosis. | 1988 Dec | To investigate esophageal involvement of scleroderma in primary biliary cirrhosis, esophageal, manometry was performed in 18 patients (16 females, two males) with primary biliary cirrhosis and in a control group of 18 subjects matched by age and sex. All patients were screened for clinical manifestations of scleroderma and for the presence of Sjögren's syndrome. Four patients had scleroderma (all of them with Sjörgren's syndrome), nine had Sjögren's syndrome without scleroderma, and five had neither scleroderma nor Sjögren's syndrome. Three patients with scleroderma had aperistalsis and diminished lower sphincter pressure. Five patients with Sjörgren's syndrome without scleroderma also had esophageal manometric disturbances. Furthermore, lower esophageal sphincter pressure (LESP) and distal mean wave pressure (DMWP) were significantly reduced in patients with scleroderma (LESP: 7.5 +/- 1.4 mmHg; DMWP: 29.5 +/- 5.9 mmHg) and in patients with Sjögren's syndrome without scleroderma (LESP: 14.8 +/- 0.8 mmHg; DMWP: 54.3 +/- 7.5 mmHg) compared to controls (LESP: 18.0 +/- 0.7 mmHg; DMWP: 83.9 +/- 5.1 mmHg). By contrast, LESP and DMWP were similar in patients without Sjögren's syndrome (LESP: 17.6 +/- 0.9 mmHg; DMWP: 78.2 +/- 10.9 mmHg) and controls. These results indicate that esophageal motility dysfunction is often present in patients with primary biliary cirrhosis who have scleroderma, and also in those with Sjögren's syndrome without scleroderma, suggesting that some esophageal motor disturbances could be related to association with Sjögren's syndrome. | |
| 3687635 | [A case of nephrocalcinosis due to Sjögren syndrome associated with renal tubular acidosi | 1987 Jul | A 39-year-old women was admitted to the hospital because of fever, lumbago and recurrent history of spontaneous stone discharge. An abdominal X-ray film demonstrated multiple calculi in the medullary positions of both kidneys and right multiple ureteral stones. Laboratory examinations showed hypergammaglobulinemia and the urine pH level was fixed around 7; nevertheless general metabolic acidosis existed. These laboratory data indicated nephrocalcinosis due to Sjögren syndrome associated with renal tubular acidosis. Right multiple ureteral stones were removed by transurethral ureteroscopy. After starting alkali therapy, neither increased nor recurrent stone formation was recognized. | |
| 3473621 | Impaired indomethacin-boosting of the defective natural killer cell activity in patients w | 1986 | As it has been shown that the natural killer (NK) cell activity is depressed and the prostaglandin (PG) metabolism is disturbed in patients with primary Sjögren's syndrome (primary SS), we examined the in vitro effect of indomethacin on the NK cell activity. The NK cell activity against K 562 target cells was measured in 20 female patients with primary SS and in 18 female normal controls matched for age. Blood mononuclear cells were incubated with indomethacin (1 microgram/ml). Median baseline NK cell activity in primary SS was 15.4% (range 5.8-50.4) versus 24.7% (range 6.2-47.4) in the controls (p less than 0.05). After in vitro exposure to indomethacin median NK cell activity in the patients was 21.2% versus 34.9% in the controls (p less than 0.02). Indomethacin-enhanced NK cell activity was significantly higher than baseline NK cell activity in both patients (p less than 0.05) and controls (p less than 0.01), but the NK cell activity of the patients was significantly less enhanced than that of the controls (p less than 0.02). We conclude that the impaired indomethacin-boosting of NK cell activity in patients with primary SS probably reflects a decreased capacity of blood mononuclear cells to produce PG in vitro. | |
| 2736824 | Evaluation of unstimulated whole saliva flow rate and stimulated parotid flow as confirmat | 1989 Mar | Unstimulated whole saliva collection (U.W.S.C.) and stimulated parotid flow rate (S.P.F.R.) are routine tests for evaluation of xerostomia. Different groups of investigators, however, use different normal values; some prefer one test (either one) more than the other. This prompted us to evaluate these tests in 81 well documented primary Sjögren's syndrome (prim. SS) patients and 188 age and sex matched normal volunteers. The U.W.S.C. as well as the S.P.F.R. were performed for 5 min. Lemon juice was used for stimulation and the saliva from the parotid gland was collected using modified Carlson-Crittenden cups. Normal individuals produced from between 0.20 and 6.81 ml/5 min (x +/- SD: 2.03 +/- 1.36 ml) of U.W.S. and from between 0.00 and 7.50 ml/5 min (x +/- SD: 1.00 +/- 1.10 ml) of S.P.F. No statistical differences were observed between the two salivary flow rates. Primary Sjögren's syndrome patients produced from 0.04 to 2.00 ml/5 min (x +/- SD: 0.47 +/- 0.50 ml) of U.W.S. and from 0.00 to 2.00 ml/5 min (x +/- SD: 0.37 +/- 0.56 ml) of S.P.F. Using the 95% confidence limit of the flow rates (U.W.S.: 1.25 ml/5 min and S.F.P.: 1.5 ml/5 min) observed in prim. SS patients, it was observed that 57% of the U.W.S. and 78% of the S.P.F values produced from normal individuals fall into that range. Based on these findings we conclude that these two tests are not useful for the evaluation of xerostomia. | |
| 3291481 | [The Ro/SS-A antigen-antibody system]. | 1988 Mar | First described in 1969 by Clark et al., the Ro/SS-A antibody system has proved most important in the evaluation of lupus patients possessing prominent photosensitive cutaneous lesions. Ro antibodies are also seen in patients with primary Sjögren's syndrome, neonatal lupus erythematosus, congenital heart block and ANA-negative LE. The most recent data of biochemical characterization have led to the supposition that these antibodies may exert a direct pathogenic effect. In this review the current knowledge concerning the Ro-antigen-antibody system is summarized. | |
| 3394094 | [Biopsy of the minor salivary glands in the differential diagnosis of Sjögren's syndrome | 1988 | The authors studied minor salivary gland morphology of 59 patients with different forms of chronic sialadenitis and Sjogren's syndrome (SS). The presence of pathomorphological changes in the salivary gland was established in chronic sialadenitis as well as in SS. However, these changes had distinctive features: total involvement in SS and mosaic patterns in chronic sialadenitis. The authors made a conclusion of the systemic involvement of the salivary glands in chronic sialadenitis. | |
| 3434282 | Minor salivary gland swelling in patient with Sjögren's syndrome. | 1987 Oct | A case of Sjögren's syndrome with an unusual minor salivary gland swelling is reported. The patient was a 63-year-old female with a chief complaint of palatal swelling and dry mouth. Clinical examination revealed diffuse swelling of the hard palate and bilateral parotid regions, and sialographic examination of the parotid gland showed evidence of sialoangiectasia. Histopathological findings of the palatal and parotid lesions revealed intense lymphoid cell infiltration replacing the salivary gland parenchyma, and scattered epimyoepithelial islands throughout the lesions. Although it is well known that major salivary gland swelling caused by marked lymphoid cell infiltration is one of the common features in patients with Sjögren's syndrome, a similar swelling in the region of the minor salivary glands is not usually apparent. | |
| 3663254 | Felty's syndrome: long-term followup after treatment with auranofin. | 1987 Sep | Five patients who had Felty's syndrome were treated with auranofin, 6 mg/day, for a period that ranged from 4 months to 2 years. All patients experienced both an improvement in articular symptoms and a normalization of the leukocyte count. Auranofin appears to be an effective treatment for Felty's syndrome, and to have a lower degree of toxicity than parenteral gold. | |
| 3316659 | Autoantibody analysis of patients with graft versus host disease. | 1987 Sep | The sera of 26 patients with graft versus host disease (GVHD) were analyzed for the presence of autoantibodies. Because the clinical spectrum of GVHD resembles some of the systemic collagen vascular diseases, particular attention was given to antinuclear antibodies and autoantibodies directed against saline soluble cellular antigens. 39% (10/26) of the patients had a positive ANA at a titer of greater than or equal to 1/80. Antibodies to double-stranded DNA were demonstrated in 4 sera (15%), to smooth muscle in 9 (41%) and to nucleoli in 6 (22%). Three sera had precipitating antibodies to saline extracts of rabbit thymus and/or bovine spleen. None of these precipitins showed lines of identity with known autoantibody systems. High titers of ANA were correlated with a previous diagnosis of acute lymphoblastic leukemia and multiple autoantibodies correlated with the severity of GVHD. | |
| 3033856 | Systemic evaluation of Sjögren-like syndrome after bone marrow transplantation in man. | 1987 May | A systematic evaluation of Sjögren-like syndrome (SLS) was performed in 68 bone marrow transplant (BMT) recipients (60 allogeneic and 8 syngeneic recipients). At day 100, the patients underwent clinical evaluation, functional salivary scintigraphy, and lip biopsy. If any findings were abnormal, the examinations were repeated annually for 3 years. Twenty-two patients with SLS and extensive chronic graft-versus-host disease (CGVHD) had abnormal scintiscan and lip biopsy at day 100. Marked keratoconjunctivitis sicca and xerostomia developed between 12 and 24 months after BMT and, thereafter, progressively decreased. Twenty-seven irradiated recipients (7 syngeneic and 20 allogeneic recipients without CGVHD) had isolated xerostomia and disturbed scintiscan but normal biopsy. Seven other patients with limited CGVHD had a lymphocytic infiltrate on lip biopsy but no SLS and a normal scintiscan. Schirmer's test, functional salivary scintigraphy, and lip biopsy allowed us to distinguish SLS from radiotherapy sequelae. As early as day 100, these 3 tests have a predictive value for SLS, one of the criteria for extensive CGVHD. | |
| 3815841 | Sjögren's syndrome and vulvar cancer. | 1987 | In the vulvar pathology surgery unit we have come across 3 patients with Sjögren's syndrome. Two of them also now show the presence of vulvar carcinoma, and one of them shows the presence of vulvar dystrophy with dysplasia. This has induced us to consider the possible connections between the 2 pathologies. All 3 of the patients, at different times and in different periods have been treated with steroids for the original disorder. The connections between the 2 affections are presumably to be found in the possible autoimmunitary genesis of the original disease and in the concomitant use of steroids. | |
| 3576444 | Periocular granuloma annulare. | 1987 Jan | A 2-year-old boy presented with a 3-month history of nontender masses involving the right superotemporal orbit and scalp. Over the three-week period before presentation he also developed nodules on his wrist, abdomen, and buttocks. The remainder of the ocular and general physical exam was unremarkable. A biopsy of the right orbital mass demonstrated stellate areas of collagen necrosis surrounded by pallisading epithelioid histiocytes and scattered multinucleated giant cells. Special stains for connective tissue mucin were positive in the areas of necrosis. The diagnosis reached pathologically and clinically were that of pseudorheumatoid nodules and granuloma annulare syndrome respectively. These two diagnostic terms have been used independently in the ophthalmologic and dermatologic literature, despite the identical histologic and clinical behaviors seen by both medical groups. A unified nomenclature system of pseudorheumatoid nodule for the histopathology of an individual lesion and granuloma annulare for the syndrome is suggested. | |
| 3787057 | [Sensory trigeminal neuropathy in systemic diseases: 4 cases with study of the trigeminofa | 1986 | Isolated trigeminal nerve affections can occur during the course of various connective tissue diseases, particularly scleroderma and mixed connective tissue lesions. Four cases are reported: a patient with systemic scleroderma, one with atrophic polychondritis, one with Gougerot-Sjögren's disease and one with atypical and frustes connective tissue lesions. The mechanisms of onset and lesional location in these neuropathies are poorly understood. A blink reflex study by electrical stimulation of the supraorbital nerve was carried out in these 4 patients to determine the site of lesions. Response was normal in 1 case suggesting a lesion of a distinct branch of the supraorbital nerve. In 2 cases the anomalies of the early response were strongly suggestive of a peripheral, truncal or radicular lesion. In the last patient the early response was normal and latencies in tardive responses of the stimulated side were in favor of a central lesion of the spinal root or spinal nucleus of the trigeminal nerve. Clinical characteristics of some reported cases of neuropathy of trigeminal nerve also appear to point to a central lesion. |