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ID PMID Title PublicationDate abstract
1339235 Vascular endothelium and lymphocyte adhesion molecules in minor salivary glands of patient 1992 Jan The aim of this study was to examine the distribution and types of adhesion molecules expressed over endothelial cells and the ligands present on lymphocytes which infiltrate exocrine glands in patients with Sjogren's syndrome. Minor salivary gland biopsies were examined from twelve patients with Sjogren's syndrome and eight normal subjects for the presence of adhesion molecules using monoclonal antibodies and an Indirect Immunoperoxidase technique. There was an increased expression of intercellular adhesion molecule-1 (ICAM-1, CD54) on endothelial cells, lymphocytes, fibroblasts and salivary gland epithelial cells. In addition we documented the expression of endothelial leukocyte adhesion molecule-1 (ELAM-1) on endothelial cells in salivary glands from patients but not the controls. Many of the endothelial cells expressing these adhesion molecules in patients with Sjogren's syndrome had the morphological appearance of high endothelial venules. V-CAM-1 was shown to be present in some of the salivary biopsies from patients with Sjogren's syndrome. Lymphocytes infiltrating salivary glands strongly express LFA-1 (CD11a/CD18) molecules. Some infiltrating lymphocytes, and most monocytes, expressed C3bi-R (CD11b/CD18) and the p150.95 (CD11c/CD18) antigens on their cell surface. The results of this study reveal the enhanced expression of vascular endothelial and lymphocyte adhesion molecules on the minor salivary glands of patients with Sjogren's syndrome. The presence of such receptors and their putative ligands indicate an important role for these molecules in the pathogenesis of Sjogren's syndrome.
9452641 Whole saliva and the diagnosis of Sjögren's syndrome: an evaluation of patients who compl 1996 Jul Sjögren's syndrome (SS) is a multisystem, autoimmune disease characterised by generalised desiccation, exocrine hypofunction and serologic abnormalities. Last year we showed that the antibodies which are quasi-specific for diagnosis of SS, anti SS-A/Ro and anti SS-B/La, were present in the saliva of patients with this disease. OBJECTIVE: To determine their presence in patients who complain of dry mouth and dry eyes. SETTING: The study was conducted at the School of Dental Medicine, SUNY at Stony Brook. PATIENTS: There were 49 patients (Mean Age = 54 +/- 13 years). TESTS: Serum was analysed for the SS and other antibodies with "Western Blot Autoantibody Strips". RESULTS: The findings showed that there was a strong correlation between the presence of the SS antibodies in the serum and the saliva. The SS antibodies were primarily found in the saliva of the patients whose resting and stimulated, whole saliva, flow rates were abnormally low. Antibodies to other autoimmune disease (lupus, scleroderma and mixed connective tissue disease) were also found in whole saliva. CONCLUSIONS: The findings in Part 1 of this study, of patients who had complained of dry mouth and dry eyes, suggest that those patients, who demonstrated low resting and stimulated flow rates and had lacrimal hypofunction, suffered from SS. The observation in this paper, that the whole saliva of these patients contains the SS antibodies, confirms this diagnosis. The data suggest that whole saliva can be used to establish the diagnosis of SS and other autoimmune diseases.
8722196 The role of molecular analyses of B-cell and T-cell clonality in the study of B-cell lymph 1996 Jan While the simple detection of B-cell clonality does not imply B-cell malignancy, comprehensive analyses of B-cell clonality are crucial to investigate the pathobiology of B-cell lymphoproliferative disorders. Our recent studies in patients with Sjögren's syndrome have highlighted how multiple molecular analyses of B-cell clonality (Southern blot, polymerase chain reaction, single strand conformation polymorphism, and DNA sequence analysis) in prelymphomatous lesions may be of great value in helping to define the stages of progression towards low-grade malignancy. The study of T-cell expansion may also be important in investigations of the pathobiology of the different stages of B-cell lymphoproliferation (fully benign, pseudolymphomatous, or definitely malignant), which may still be T-cell-and antigen/ autoantigen-dependent.
8610217 Primary Sjögren's syndrome (SS) and malignant lymphoma. A retrospective cohort study of 5 1995 The aim of this study was to assess the prevalence of malignant lymphomas in patients with long-standing primary Sjögren's syndrome (pSS). We retrospectively studied a cohort of 55 patients with pSS over a mean follow-up period of 12 years. Five patients (9%) developed malignant lymphoma. The interval between the diagnoses of SS and lymphoma ranged from four to 12 years (mean = 6.5 years). The lymphoma arose in the lymph nodes in two cases, the parotid gland in one case, the lacrimal gland in one case, and the lung in one case. All five cases were B-cell low-grade lymphomas. Among our SS patients, those with extraglandular manifestations and/or a mixed cryoglobulin were at increased risk for lymphoma development. Secondary lymphoma carried a poor prognosis in our study. Three of the six SS patients who died during the follow-up period had lymphoma.
7535294 Hepatitis C virus and Sjögren's syndrome. 1994 Nov Viral infection has been suggested as a possible cause of Sjögren's syndrome. After we had noted Sjögren's syndrome in several patients infected with hepatitis C virus (HCV), we set up a prospective study to investigate the association of Sjögren's syndrome and HCV liver disease. We studied 10 patients with primary Sjögren's syndrome and observed four with HCV infection. A striking association between HCV infection and Sjögren's syndrome was found; however, a direct link could not be proved.
8261670 IgG2 deficiency in primary Sjögren's syndrome and hypergammaglobulinemic purpura. 1994 Jan Total IgG and IgG subclasses were studied in 34 patients with primary Sjögren's syndrome and 4 with hypergammaglobulinemic purpura. Total IgG was elevated in 30/34 patients with Sjögren's syndrome. IgG1 increase was responsible for the main part of total IgG increase, contrasting with low levels of IgG2. The difference in IgG1/IgG2 ratio between 38 patients as a group and 40 normal controls was statistically highly significant, but was not seen in all patients. Six patients had markedly low levels of IgG2, but only two had severe repeated respiratory infections. These observations probably reflect selective autoantibody restriction to the IgG1 subclass. We conclude that patients with Sjögren's syndrome may be IgG2 subclass deficient despite elevated levels of total IgG, but also that such deficiency in most instances does not cause a tendency to infections. IgG subclass analysis may be of value to characterize polyclonal IgG increase, since IgG1 subclass predominance often indicates autoimmune disease.
8114302 [Association of autoimmune disease in primary biliary cirrhosis]. 1994 Jan Association of autoimmune disease in patients with primary biliary cirrhosis (PBC) was described. Forty-one patients with asymptomatic PBC (a-PBC) and 13 patients with symptomatic PBC (s-PBC) were examined. The overall prevalence of autoimmune disease in association with PBC was 63%. There was no significant difference in the frequency of autoimmune disease association between a-PBC (63.4%) and s-PBC (61.5%). The most frequent complication was Sjögren's syndrome (31.7% in a-PBC and 38.5% in s-PBC). Raynaud's phenomenon was seen in 7 patients (12.2% in a-PBC and 15.4% in s-PBC). Five of these 7 patients showed the CREST syndrome having the anti-centromere antibody. Association of 3 polyarthritis, 2 autoimmune hepatitis, 2 Hashimoto's disease, 1 systemic lupus erythematosus, 1 polymyositis and 1 temporal arteritis was seen in a-PBC patients.
8531343 [Current status and problems in the treatment of patients with Sjögren's syndrome]. 1995 Oct Current status of treatment of patients with Sjögren's syndrome in Japan was reviewed. Although the number of patients with the syndrome has been increasing due to the recent progress in diagnosing and understanding of the disease, treatment of patients still remains unsatisfactory. However a wide variety of methods have become available in the management of patients who have significant dryness of eye and mouth. This includes artificial tear, artificial saliva, moisture shields of glasses, and many other medical and nonmedical preparations. All patients with sicca complaints should receive therapy of tear replacement and intensive oral hygiene. New potential therapeutic approaches for treatment of Sjögren's Syndrome was also reviewed.
1496163 Ocular manifestations of Sjögren's syndrome: keratoconjunctivitis sicca. 1992 Aug As part of the triad of Sjögren's syndrome (SS), keratoconjunctivitis sicca (KCS) can be found in virtually all SS patients. This term emphasizes not only the reduced tear production but also the pathologic changes in the epithelial cells of the ocular surface. The symptoms of dry eye may vary from one patient to another depending on the severity of dryness, the ability of the diminished tear film to moisten the ocular surface, and the patient's tolerance for ocular discomfort. Treatment of the dry eyes is remarkably effective, and most patients benefit from moisturizing eyedrops to replace their own deficient tears. Major issues include the pathogenesis of decreased tear flow and ocular surface disturbances, as well as the roles of wound healing agents and anti-inflammatory medications in KCS therapy.
7495348 Primary Sjögren's syndrome in men. 1995 Sep OBJECTIVE: To describe the clinical expression of primary Sjögren's syndrome (SS) in men, focusing on extraglandular manifestations (EGM) and serological markers of disease. METHODS: In a cross sectional and comparative study, adult men with primary SS were identified from a cohort study on SS, and 26 age matched adult women with primary SS were selected as a control group. All patients met the European classification criteria for SS. They were compared for demographic, clinical and laboratory findings. RESULTS: Thirteen men with primary SS were identified. Mean age at onset was 39 (SEM 4) years and mean duration of disease was 7.8 (1) years. Sicca complex or parotitis was the presenting feature in eight patients (61.5%), and an EGM in five (38.5%). During the course of the disease, EGM were present in 12 patients (92%), polyarthralgias and lymphopenia being the most frequent (38.5% each). Rheumatoid factor was positive in 73% of patients, antinuclear antibodies in 85%, anti-(SS-A) in 62%, and anti-(SS-B) in 46%. No statistical differences in the frequency of EGM or in the presence of autoantibodies were observed between men and women. However, men patients were more likely to have EGM. CONCLUSION: Primary SS in men is an uncommon condition with clinical and serological characteristics similar to those observed in women. Sex hormones may be incriminated in the pathogenesis of SS. However, it remains poorly understood whether sex hormones play a major role in the severity of disease and have any importance with regard to treatment.
7844906 [A case of multiple nodular pulmonary amyloidosis associated with Sjögren's syndrome]. 1994 Oct A 58-year-old female, who had complained of dryness of the conjunctiva and mouth for nine years, was admitted to our hospital because chest X-ray films revealed increases in both the size and the number of pulmonary nodules in comparison to four years earlier. Histological findings of the salivary gland biopsy confirmed a diagnosis of Sjögren's syndrome. An open lung biopsy was performed. Histologically, the pulmonary nodules consisted of homogeneous, acellular eosinophilic materials, which were identified as amyloid by Congo red staining. Immunohistochemical examination showed the amyloid to be composed of AA protein. There were no deposits in other organs. Peribronchial lymphocytic infiltration associated with Sjögren's syndrome was present. Nodular pulmonary amyloidosis with Sjögren's syndrome is very rare condition. To our knowledge, this case is only the ninth to be reported in the literature.
8437873 Pathology of labial salivary gland cellular aggregates in Sjögren's syndrome. 1993 Jan Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disease in which lymphocytic infiltration of the lacrimal and salivary glands is the hallmark of disease diagnosis. The present study was conducted to identify developmental features of labial salivary gland histopathology to permit earlier diagnosis of SS patients with borderline biopsies. Control subjects were chosen on the basis of clinical presentation consistent with SS, but whose biopsies did not meet current focus score criteria. Intraglandular connective tissue and diffuse cellular infiltration were significantly greater in SS patients than in controls. Glands in both groups had small cellular aggregates (10 to 50 cells/100 microns2), but those in SS patients were more numerous, larger, and contained more lymphocytes, plasma cells, and active fibroblasts. The large inflammatory foci characteristic of SS appeared to be formed by the enlargement and merging of these aggregates. This suggested aggregate formation was the earliest stage of pathology. Therefore, a comprehensive analysis of these cellular aggregates in borderline or negative biopsies could identify at-risk patients and lead to earlier diagnosis and intervention in the disease.
8355184 Rapamycin, a potential disease-modifying antiarthritic drug. 1993 Aug Rapamycin (RAPA), a potent immunosuppressive agent that prevents organ graft rejection in animal models of transplantation, possesses a mechanism of action different than that of cyclosporin A and FK-506. In this study, the pharmacological activity of RAPA in a variety of immune and inflammatory models was assessed in order to define better its potential utility as an antiarthritic agent. RAPA inhibited T cell-mediated inflammation in mouse methylated bovine serum albumin-induced delayed-type hypersensitivity (ED40 = 4.7 mg/kg p.o.) and produced oral ED50 of 2.0 mg/kg against developing adjuvant arthritis in rats (3-day dosing schedule) and 9.5 mg/kg in established adjuvant arthritis in rats (daily dosing schedule). In both models of adjuvant arthritis, effects of RAPA were maintained even after cessation of drug dosing. In contrast, after discontinuation of cyclosporin A (5- and 10-mg/kg doses), disease activity returned. RAPA was also effective in another T cell-mediated model, experimental allergic encephalomyelitis (ED50 approximately 5 mg/kg p.o.). At higher doses, RAPA significantly inhibited carrageenan paw edema in rats, a model of acute inflammation (ED40, 56 mg/kg p.o.), without increasing serum corticosterone levels. In this model, doses approximately 10 to 20 times greater than active doses in T cell-mediated models were required. RAPA at 1 to 50 microM did not inhibit in vitro human synovial phospholipase A2 or 5-lipoxygenase and cyclo-oxygenase activity in the human blood leukocyte assay. The total profile of RAPA suggests that it may be effective in the treatment of rheumatoid arthritis, multiple sclerosis and other autoimmune diseases.
8302055 Aortic root replacement with pulmonary autograft in children. 1994 Feb Between September 1988 and February 1993, 14 patients whose ages ranged from 3 months to 16 years (mean 11.1 +/- 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1% (95% confidence limits 0% to 21%). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation. Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft. The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiographic follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.
9208582 [The pathology of labial salivary gland in the diagnosis of Sjogren's syndrome]. 1996 Feb To explore the sensitivity and specificity of the pathology of labial salivary gland in the diagnosis of Sjogren's syndrome (SS) and its specific pathological changes in SS, 100 labial salivary gland specimens were observed and assayed in our hospital. The results indicated that there was no significant difference in acinar atrophy, duct dilatation and lymphocytes infiltration between primary SS (1 degree SS) and secondary SS (2 degrees SS) patients. But those changes had significant difference between 1 degree SS and non-CTD patients (P < 0.01). It was also found in our study that Chisholm grade IV was achieved in 78.57% 1 degree SS specimens, 65.22% 2 degrees SS specimeus and 22.22% in non-CTD specimens, respectively. According to Chisholm standard, the sensitivity and specificity of pathology in the diagnosis of these SS patients was 78.8% and 77.1%. Our patients were still classified by using Chisholm standard. If focal lymphocytes infiltration was used as the single criterion to SS diagnosis, its specificity was not high. In the diagnosis of SS authors should consider the pathologic changes of salivary gland as well as the clinical information in order to avoid misdiagnosis.
1319698 Constitutive production of angiotensin converting enzyme from rheumatoid nodule cells unde 1992 Jun Angiotensin converting enzyme was assayed in serum free culture supernatants from unstimulated rheumatoid nodule cells. Angiotensin converting enzyme was released spontaneously and the angiotensin converting enzyme derived from rheumatoid nodule cells was suppressed in a dose and time dependent manner by the protein synthesis inhibitor cycloheximide. These data suggest the constitutive de novo synthesis of angiotensin converting enzyme by rheumatoid nodule cells.
8990706 Multiple basaloid cell hamartoma with alopecia and autoimmune disease (systemic lupus eryt 1996 Nov We report a 22-year-old Japanese woman with multiple basaloid cell hamartoma with alopecia and autoimmune disease (systemic lupus erythematosus). She presented with infiltrated large annular, brown-violet, erythematous plaques with atrophic areas in the center on her right cheek, left abdomen and left knee. She also had progressive multiple follicular keratotic papules on her scalp, face, neck, and both axilla and hair loss from her scalp, eyebrow, and axilla. Serologically, rheumatoid factor (+ + +), rheumatoid arthritis hemagglutination test (x1280), and anti-nuclear antigen (x160) were positive. Histological findings of the annular lesion showed liquefaction degeneration of basal cells, lymphocytic infiltration around hair follicles and capillaries, and panniculitis with lymphoid cell infiltration, which was diagnosed as lupus erythematosus profundus. The histological findings of multiple follicular papular lesions of the scalp and neck showed aggregations of basaloid cells, partially with hair-bulb-like structures, which was diagnosed as trichoepithelioma. Taken together, the histogenesis of multiple basaloid cell hamartoma is thought to share the same basis with autoimmune disease.
8752528 [Development of collagen vascular diseases and production of autoantibodies in HTLV-I env- 1996 May Human T lymphocyte virus type I (HTLV- I) is now known to be associated with a number of diverse clinical disorders, not only adult T cell leukemia but also HTLV- I associated myelopathy/tropical spastic paraparesis, HTLV- I -associated arthropathy, HTLV- I uveitis, and probably Sjögren's syndrome, T cell alveolitis, polymyositis, and infective dermatitis. To investigate virus-host interactions and pathogenetic mechanisms in these diverse disorders, inbred rat, which is susceptible to HTLV- I infection and develops HAM/TSP-like disease by HTLV- I infection, was used as a host of HTLV- I gene transfer model. HTLV- I LTR-env-pX-LTR construct was injected to rat ova, and two lines of the transgenic rat (env-pX rat) were established. Both lines of env-pX rats expressed HTLV- I env and pX genes in various tissues, and developed a wide spectrum of collagen vascular diseases, including chronic destructive arthritis similar to rheumatoid arthritis, necrotizing arteritis mimicking polyarteritis nodosa, myositis, myocarditis, and chronic sialoadenitis and dacryoadenitis resembling Sjögren's syndrome in humans. Thrombosis and thymic atrophy were also observed. These rats showed hyper-gamma globulinemia and a number of autoantibodies, including high titered rheumatoid factors, anti-dsDNA antibodies and anti-cardiolipin antibodies were presented in the serum. Results suggest that the HTLV- I env-pX gene may play a pathogenic role in development of collagen vascular diseases associated with autoimmune phenomenon. The env-pX rat appears to be a suitable animal model for elucidating pathogenetic mechanisms implicated in HTLV- I -induced diseases and also in various collagen vascular diseases of unknown etiology in humans.
7892658 Halifax clamps: efficacy and complications in posterior cervical stabilization. 1995 Feb BACKGROUND: Trauma, neoplasia, rheumatoid arthritis, Down's syndrome, and inflammatory conditions are well-known causes of spinal instability. The Halifax clamp is a method of posterior cervical stabilization that is attached to the adjoining laminae and tightened until no movement between involved vertebrae is possible. Our experience with cases that have utilized Halifax interlaminar clamps, their results and complications are presented. METHODS: We conducted a retrospective review of patients with spinal instability in whom Halifax interlaminar clamps were utilized for posterior spinal stabilization. RESULTS: Over a 5-year period, 38 patients in our institution underwent posterior stabilization of the cervical spine utilizing the Halifax interlaminar clamp. The patients represent a mix of motor vehicle- and sports-related traumas, rheumatoid subluxation, and a single cancer patient. Average follow-up has been 29 months (range 3 to 36 months). Thirty (78.9%) patients have had good results (i.e., stable fusion and regression of symptoms) and no complications. Three patients from our institution, and a fourth patient referred to us postoperatively, developed complications related to the Halifax clamps. Four patients died from unrelated causes. CONCLUSIONS: Halifax interlaminar clamps are a safe and effective method for posterior stabilization of various causes of cervical spinal instability.
7548882 Pathogenesis aspects of arthrosis in histopathological and electronmicroscopical studies o 1994 Jul Twenty-five biopsies of arthrosic cartilage with radiological correspondence, arthro, sic cartilage under study for Rheumatoid Arthritis (RA) and posttrauma cartilage as control-were examined using histopathological (HE, VG, PAS-Alcian, Gömöri, Safranine O) and electronmicroscopical techniques. The arthrosic cartilage with radiological correspondence shows superficial and deep fissures, perichondrocytic gaps and modified reticulino-collagenic network at the histopathological examination. At the level of synovia-cartilage junction, we found some villous aspects of the synovia desquamating in the proximity of the affected cartilage. The investigated arthroses for RA presented some destructive fibrous modifications of the synovia similar to rheumatoid synovitis and associated with some dystrophic chondrocytic alterations. The ultrastructural affection was severe leading to cellular degeneration. The immunologically-studied arthroses for RA had seric pathologic values regarding: circulating immune complexes (CIC) (mean = 67.08 +/- 1.45 U), Ig.M(mean = 358 +/- 3.02 UI/ml) and anti collagen antibodies (mean = 409.9 +/- 0.42 U). The synovial depletion of complementary fraction C3(mean = 42.3-1 mg%) as against the normal seric level (mean = 63.07 +/- 0.49 mg%) suggests an immune synovitis. Correlation of immunomorphopathological data emphasize that arthrosis coexists with a secondary synovitis following a primitive degenerative process and allows arthroses under study for RA to be separated from other degenerative rheumatism diseases.