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ID PMID Title PublicationDate abstract
8050191 Rearrangement of the rheumatoid factor-related germline gene Vg and bcl-2 expression in ly 1994 Aug Of 250 patients with Sjögren's syndrome (SS) (190 with 1 degree SS and 60 with 2 degrees SS), 60 patients demonstrated lymphoproliferative disorders (LPD): 41 patients had monoclonal gammopathy (MG), 4 had pseudolymphoma, 3 had in situ monoclonal lymphoproliferation, and 12 had malignant lymphoma. Rearrangement of the rheumatoid factor (RF)-related germline gene Vg (associated with the monoclonal RF SF18/2) was demonstrated in 24 of 50 genomic DNAs from the peripheral blood leukocytes of SS patients. It was found in none of 20 DNAs from normal subjects. This suggests an almost 50% incidence of a germline Ig gene rearrangement in SS patients without clinical MG. Lymphocytes composing the lymphoepithelial lesion (LEL) in the major salivary glands of SS patients expressed the oncogene bcl-2 protein in 4 out of 6 patients. The progression of SS from benign to malignant lymphoproliferation may be related to suppression of apoptotic death by bcl-2. These findings suggest that (i) RF clones are activated in SS with little or no somatic mutation resulting in monoclonal proliferation and (ii) the LEL in the salivary gland is one site for monoclonal B cell proliferation and emerging malignant lymphoma.
8509804 Occurrence of primary biliary cirrhosis, CREST syndrome and Sjögren's syndrome in a patie 1993 May We report the occurrence of three autoimmune diseases (primary biliary cirrhosis, CREST syndrome and Sjögren's syndrome) in a patient with HTLV-I-associated myelopathy (HAM). The patient had the depressed cell-mediated immune responses but the infiltration of CD8-T cells was found in the cerebrospinal fluid and liver. The clinical and immunological features of this case are similar to those of chronic graft-versus-host disease.
7561120 Limited TCR repertoire of infiltrating T cells in the kidneys of Sjögren's syndrome patie 1995 Oct 15 To analyze the mechanism of interstitial nephritis in patients with Sjörgren's syndrome (SS), we examined the TCR repertoire of infiltration T cells in kidney, labial salivary glands, and PBLs using a PCR. The repertoire of the TCR V beta gene on infiltrating T cells from the kidneys of SS patients was more restricted than those on infiltrating T cells in labial salivary glands and PBL. The TCR V beta 2 gene was expressed predominantly in six of seven (86%) SS patients. Junctional sequences of cDNAs encoding the V beta 2 gene on infiltrating T cells in the kidneys of five SS patients showed that some of the cells expanded clonally, indicating Ag-driven stimulation rather than superantigen-induced proliferation. The same V beta 2 clones in the kidney were not detected in labial salivary glands of the same SS patients; the conserved amino acid (arginine at position 96) in the CDR3 region of the V beta 2 gene was found at a frequency of 48.0% in the kidney, whereas it was detected in only 15.4% of the clones in lips. In conclusion, these findings suggest the possibility that T cells that infiltrate the kidneys of SS patients with interstitial nephritis might recognize different autoantigens than those that infiltrate labial salivary glands.
7991440 Maternal collagen vascular disease associated with fetal heart block and degenerative chan 1994 Jul We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insufficiency occurred following placement of an endocardial pacing lead, and severe mitral insufficiency occurred acutely at 2.5 months of age. Both valves were noted at surgery to have fibrotic and calcific chordae tendineae that had ruptured from their papillary muscles. An inflammatory reaction within the fetal heart related to transplacental passage of Sjögren's antibody was likely responsible.
7564265 Expression of CD60 on multiple cell lineages in inflammatory synovitis. 1995 Sep BACKGROUND: CD60 is a recently described T cell subset marker that is expressed on the surface of most T lymphocytes in synovial tissue and fluid and on a smaller proportion of peripheral T cells. Activation of T lymphocytes can be triggered through CD60. CD60 is also expressed by neuroectodermally derived cells in thymic epithelium and in skin. EXPERIMENTAL DESIGN: Immunohistologic analysis of CD60 expression in synovium and thymus was performed using formalin-fixed tissue samples. Nonlymphoid cell lines grown from similar tissues were analyzed by flow cytometry. RESULTS: CD60 was readily identified in formalin-fixed, paraffin-embedded tissues. Simultaneous examination of CD60 distribution and cell morphology demonstrated that, in addition to its presence on T cells, CD60 was also expressed by a variety of nonlymphoid cells in synovium, including synovial lining cells, vascular endothelium, and dendritic-appearing cells deep within synovial tissue. Synovial tissue expression of CD60 was similar in rheumatoid arthritis and in other forms of inflammatory arthritis. In addition, it was strongly expressed by giant cells in pigmented villonodular synovitis. Surface expression of CD60 was detected by flow cytometry on cultured synoviocytes and on other CD60+ nonlymphoid cells, thus excluding adsorption of CD60 shed by T cells as a sufficient explanation of the immunohistologic findings. CONCLUSIONS: These results define the T cell-activating CD60 determinant as a broadly distributed Ag within synovial tissue, with a possible functional role in the activation of a variety of cellular populations. CD60 may also be a marker for previously undescribed cell subsets in the synovial compartment, possibly including a cell population of neuroectodermal origin.
8163678 Serological and genetic characterization of a human monoclonal immunoglobulin G anti-DNA i 1994 Apr This study analyzed the distribution of an idiotype, B3-Id, in patients with active SLE, classified according to organ involvement, normal controls, and other autoimmune rheumatic diseases. A polyclonal anti-idiotype was raised by immunizing a rabbit with a monoclonal IgG anti-double-stranded (ds) DNA antibody, B3, generated from a patient with SLE who had active arthritis. The idiotype is present on the lambda chain and is at or near the binding site for double-stranded DNA. The lambda chain, which was characterized by nucleotide sequencing, was 90% homologous to the V lambda 2.1 germline, which is known to be involved in coding for nephritogenic anti-DNA antibodies carrying the 8.12 idiotype. There were four changes to positively charged amino acids, known to be involved in DNA binding, in the complementarity determining regions of B3 lambda chain compared with a non-DNA binding, 8.12 positive antibody, PV11. Only one change to a positively charged amino acid occurs in the heavy chain of B3, which is 93.5% homologous to VH-26. The B3-Id was present on IgG antibodies in the serum of 20% of patients with SLE but was not found in the normal controls. Within the SLE group, there is a statistically significant association of B3-Id on IgG in the arthritis group (42%) compared to the other manifestations (9%) (P < 0.001). In four B3-Id-positive SLE patients tested serially, the level of B3-Id reflected the arthritis disease activity more closely than the overall disease activity (P < 0.05). The B3-Id was also present on IgM antibodies in one third of patients with rheumatoid arthritis. This idiotype is the first to be derived from a human monoclonal anti-DNA antibody of the IgG class, the isotype associated with active disease. Sequence analysis shows that positively charged amino acids on the lambda chain may contribute to DNA binding.
8479324 Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a 1993 Mar In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.
8934920 Current treatment modalities of oral problems of patients with Sjögren's syndrome: caries 1996 Apr Prevention of dental caries in patients with hyposalivation due to Sjögren's syndrome requires increasing host resistance and decreasing cariogenic organisms and their substrate. Although plaque control by scrupulous oral hygiene is important, particularly from a periodontal perspective, and restriction of dietary sucrose intake can limit caries, the most successful therapeutic and preventive measure has been the topical application of fluoride to the tooth surface, by the dentist, dental hygienist, dental auxiliary, and the patient. Studies on schoolchildren with normal salivary function who used fluoride dentifrices have shown that efficacy in caries prevention depends on (1) the concentration of fluoride used, (2) the frequency with which it is applied, and, to a certain extent, (3) the specific fluoride compound used. Controlled clinical studies are lacking, however, on patients with hyposalivation due to Sjögren's syndrome, and only limited data are available from patients with radiation-induced hyposalivation. Obviously it is not possible to run placebo control groups; nevertheless, there have been no head-to-head comparisons of fluoride rinses, or of stannous fluoride, acidulated phosphate fluoride, or sodium fluoride gels, and thus no single protocol can be recommended. Accordingly, different centers have used these products interchangeably--for example, selecting a rinse regimen if patients complain of gagging when using a gel applied in a tray, or if the cost of the tray is prohibitive. Recent innovations that show promise for treatment of high-caries-risk patients with hyposalivation are the use of fluoride rinses in combination with chlorhexidine rinses or gels and the professional application of high-concentration chlorhexidine varnishes to the teeth.
8610577 Texture analysis of sonographic features of the parotid gland in Sjögren's syndrome. 1996 Apr OBJECTIVE: The purpose of this study was to develop a method for quantitative analysis of the sonographic features of parotid glands as a noninvasive tool for the diagnosis of Sjögren's syndrome. SUBJECTS AND METHODS: Sonographic texture analyses were performed on the parotid glands of 44 patients with Sjögren's syndrome, 83 healthy volunteers, and 17 patients with chronic parotitis, using a fast Fourier transform program. RESULTS: Texture analysis of sonographic studies of the parotid gland using a Fourier transform showed that the sum of the normalized radial power spectrum in the low-spatial-frequency region (S value) of the parotid gland was significantly higher (p < .0001) in the patients with definite Sjögren's syndrome [6.70 +/- 2.13 (x10(5))] than in the 72 age-matched normal volunteers [3.25 +/- 1.08 (x10(5))]. However, patients with probable Sjögren's syndrome showed S values [3.92 +/- 1.88 (x10(5))] similar to those of the controls. On the other hand, SDs of the echo levels in the parotid gland showed significantly greater (p < .0001) values in patients with definite (4.63 1.07) and probable (4.53 1.47) Sjögren's syndrome than in the normal controls (3.30 0.76). Discriminant analysis showed that a combination of these two distinctive values increased diagnostic accuracy to 96.9%. Furthermore, S values and SDs correlated well with the qualitative grading of sonographic features and with the gradings of sialography. CONCLUSION: The system we describe for texture analysis of sonographic images is useful in the diagnosis of Sjögren's syndrome.
9082596 [The comparative clinico-morphological characteristics of chronic parotitis in sarcoidosis 1996 Common and differential clinical and morphological signs of chronic parotitis, parotid gland sarcoidosis and Sjögren's disease are outlined. 5 cases of sarcoidosis and 28 cases of Sjögren's disease served as illustration.
8763101 [Fatal infectious complications in 2 patients with adult onset Still disease]. 1996 If adult Still's disease (ASD) can sometimes lead to severe destructive joint lesions and to various systemic manifestations, life-threatening complications are very rare. However, a long-term and high-dose corticosteroid therapy is often required to control the disease, with frequent corticodependence. Some authors have proposed methotrexate as a second line drug for ASD, that could permit a corticosteroid sparing effect. We report two cases of acute fatal infectious complications--legionella pneumonitis and multiple brain abscess caused by Nocardia asteroides--in two patients treated for ASD with both corticosteroids and methotrexate. These two cases raise the problem of the immunodepression induced by this combination therapy and point out the difficulties in aggressive forms of ASD.
8869222 Recurrent psoriatic onychoperiostitis induced by hydroxychloroquine. 1995 Dec Synthetic antimalarial agents can cause exacerbation of latent or patent psoriatic skin lesions. A case of psoriatic onychoperiostitis precipitated by hydroxychloroquine therapy is reported. The patient had primary Sjögren's syndrome, raising questions about the incidence and causation of the ungual abnormalities associated with this condition.
8674565 Gynaecological aspects of primary Sjogren's syndrome. 1995 Nov Female patients affected with Sjogren's Syndrome (SS) frequently describe symptoms such as vaginal dryness and dyspareunia; however, only a few controlled studies have regarded clinical involvement of the female external genitalia. OBJECTIVE: The present study was undertaken in order to: (1) Evaluate the involvement of external genitalia in a large number of female patients affected with primary SS (pSS) by semi-quantitative methods covering subjective symptoms and clinical evaluation. (2) Compare pSS patients with a matched healthy control group (pre- and post-menopausal women were separately studied). (3) Correlate the gynaecological involvement with salivary and lacrimal abnormalities in pSS patients. METHODS: We evaluated 36 patients with primary SS (18 pre- and 18 post-menopausal women) and 43 healthy controls using a questionnaire regarding vulvar and vaginal dryness and a complete gynaecological examination. Subsequently, three scores related to vulvar and cervical status plus a global score were obtained. In primary SS patients, salivary and lacrimal involvement was also evaluated. RESULTS: Dyspareunia was present in 61% and vaginal dryness in 55% of SS patients versus 39% and 33% of healthy controls. No significant differences regarding gynaecological scores were found between SS patients and controls, in both pre- and post-menopausal women, nor correlation was observed between gynaecological and lacrimal or salivary involvement. CONCLUSIONS: Our data suggest that although SS patients frequently complain of dyspareunia and vaginal dryness they do not greatly differ from healthy subjects in regard to some major gynaecological aspects.
8136465 Aberrant cellular localization of rubella viral genome in patients with adult Still's dise 1993 The rubella virus (RV) genome was detected using polymerase chain amplification techniques in several peripheral blood cell populations in patients with adult Still's disease (ASD) and normal controls (NC), including mononuclear cells (PBMC), B-cells, T-cells, monocyte/macrophages, and polymorphonuclear leukocytes (PMN). Five of 6 ASD patients and 3 of 6 NC subjects had detectable RV genome. Viral genomic load was significantly higher in ASD than in NC subjects (4.4 fold higher, p = 0.03). Interestingly, a differential cellular distribution of viral genome was observed between ASD and NC individuals. RV genome was detected more frequently in the PBMCs of ASD (5 of 6) patients compared to 2 of 6 NC. The viral genome was more localized to the PMN compartment equally in ASD and in NC subjects. On further cellular analysis, RV genome was detected in B-cells and macrophages but not T-cells in one patient. Existence of a differential viral genomic reservoir between ASD and NC suggests that this may play a role in the pathogenesis of disease manifestations and may reflect the inability to clear latent virus.
1404021 Vanishing syndromes. 1992 Jul Certain syndromes develop because of regression or shrinkage of essential pathways. This account draws attention to six conditions in which different mechanisms and pathways give rise to a scorched-earth policy and to strange clinical syndromes.
1624809 Production of monocyte chemoattractant protein-1 by inflamed synovial tissue and cultured 1992 Jul 15 This study analyzes the expression of monocyte chemoattractant protein-1 (MCP-1) by inflamed synovial tissue and defines its regulation in cultured synoviocytes. Synoviocytes from patients with rheumatoid arthritis and osteoarthritis express the 0.7-kb MCP-1 mRNA. Stimulation of synoviocytes with IL-1, TNF-alpha, LPS, platelet-derived growth factor, and transforming growth factor-beta-1, but not with basic fibroblast growth factor causes a marked increase in MCP-1 mRNA levels. Expression of the MCP-1 gene is inducible by activators of the protein kinase A (cAMP) and C (PMA) signal transduction pathways and is differentially regulated by the steroids dexamethasone and retinoic acid. Cultured synoviocytes de novo synthesize 12-, 15-, and 15.2-kDa MCP-1 proteins, which increase after stimulation with IL-1. Synovial tissues from donors without joint disease and from patients with rheumatoid or osteoarthritis were analyzed for MCP-1 mRNA expression by in situ hybridization. In these samples MCP-1 mRNA expressing cells were predominantly found in the sublining cell layers, whereas specimens of normal synovial tissue contained only few positive cells. These results identify synoviocytes as a source of MCP-1. Its expression is controlled by peptide regulatory factors that are known to be present in arthritic joints. Detection of cells producing MCP-1 mRNA in synovial tissues from patients with arthritis shows that this gene is expressed in vivo and suggests that MCP-1 can play a role in recruiting monocytes in joint inflammation.
8712887 Salivary eicosanoid concentration in patients with Sjögren's syndrome. 1996 Mar OBJECTIVE: To investigate eicosanoid concentrations in the saliva of patients with primary Sjögren's syndrome (SS). METHODS: Whole mixed saliva of 36 subjects was assayed for eicosanoid concentrations using a radioimmunoassay. Patients with primary SS having positive lip biopsy served as the study group; their results were compared with data from patients with dry mouth and negative lip biopsy (dry mouth group), and with a group of normal healthy controls. RESULTS: Concentrations of thromboxane B2 were significantly (p < 0.01) increased in 18 patients with primary SS compared with 10 patients with dry mouth and eight healthy normal controls (1.95 (SD 0.51) ng/ml saliva compared with 0.52 (0.1) ng/ml and 0.3 (0.1) ng/ml, respectively). Similarly, prostaglandin E2 concentrations were also significantly increased (p < 0.01) in 11 patients with primary SS compared with five patients with dry mouth and eight normal controls (3.75 (0.82) ng/ml saliva compared with 0.32 (0.1) ng/ml and 0.41 (0.1) ng/ml, respectively). CONCLUSION: Salivary concentrations of eicosanoids are significantly increased in patients with primary SS, and this may prove helpful in the diagnosis of this disease.
8531376 [Sjögren's syndrome associated with non-Hodgkin's lymphoma]. 1995 Oct Sjögren's syndrome (SS) is characterized by an increased risk of development of a non-Hodgkin's lymphoma (NHL). In this paper, the clinical, histological and molecular biological topics, with a brief account of two cases of NHL, in SS are discussed. Low grade NHL, typically monocytoid B cell lymphoma related to mucosa associated lymphoid tissue (MALT) lymphoma, is the topic of NHL in SS. B cell clonal expansion has been detected by DNA analysis of immunoglobulin gene in non-lymphoma patients, as well as, in NHL patients in SS. Molecular biological analysis, especially detection of fusion gene of IgH and bcl-2, t(14;18), using PCR method, can help to detect early onset of NHL in SS. A better understanding of NHL in SS is needed to develop a co-operative research of large mass study.
8052753 [Osteomalacia of renal origin disclosing Gougerot-Sjögren syndrome]. 1994 Jan Latent renal tubular diseases are common in Sjögren's syndrome but rarely complicated by osteomalacia. The authors report a 28 years old woman's case where osteomalacia reveal the disease. In the other hand, pseudo-lymphoma is suspected from radiographic features. According to several studies, the authors review different types of renal diseases and describe different physiopathological hypotheses about bone involvement and pseudo-lymphomas.
1496161 Antinuclear antibodies in Sjögren's syndrome. 1992 Aug The definition and characterization of the reactivity of ANA in patients with SS are greatly improved with the current advances in cellular and molecular biology. Based on the characteristic autoantibody profiles in different systemic rheumatic diseases and the nature of the defined autoantigens, it has been proposed that the polyclonal autoimmune response is induced and maintained through an antigen-driven mechanism. The autoantigens are presented in the cell as components of large particles or structures composed of protein-protein or RNA-protein complexes; however, the role of these autoantibodies in the etiology and immunopathogenesis of SS remains to be determined.