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ID PMID Title PublicationDate abstract
7940341 [The x-ray changes in the parotid salivary glands of patients with Sjögren's disease]. 1994 The data obtained upon sialography of the parotid glands (PG) in 28 patients with Sjogren's disease (SD) were compared to clinical SD manifestations, PG regional circulation and histological picture of the labial salivary glands. 24 patients exhibited classical sialography characterized by sialoectasias, enlarged ducts, obscure outlines of the latter. 4 sialograms displayed the duct narrowing, noncontrast parenchyma outlines, unclear images of sialoectasias. Severe xerostomia, marked dryness of the vermilion border, rare enlargement of the salivary glands and parotitis recurrences occurred more frequently in patients with narrow efferent PG ducts. These changes are attributed to vascular sclerosis, devascularization, stromal sclerosis and parenchymal PG atrophy.
8261782 Effect of topical cyclosporin A on conjunctival T cells in patients with secondary Sjögre 1993 Nov The effect of topical cyclosporin A on conjunctival T cells was studied in nine patients with secondary Sjögren's disease. Patients had conjunctival biopsies performed before and after a 6-week course of topical cyclosporin. Epithelium and substantia propria in the Sjögren's patients before treatment showed significantly more CD4+ cells than specimens taken from nine age- and sex-matched controls. Following treatment with topical cyclosporin, there was a significant reduction in the number of CD4+ cells in both the conjunctival epithelium and substantia propria. Despite the fact that the treatment resulted in immunopathological improvement, the clinical benefit was not as favorable. Our results suggest that topical cyclosporin may have a local immunosuppressive effect on the conjunctiva in patients with Sjögren's disease.
8934919 Is sialography effective in diagnosing the salivary component of Sjögren's syndrome? 1996 Apr Parotid sialography has been used for many years as a means of assessing salivary glands in Sjögren's syndrome (SS), and it is occasionally used as a diagnostic criterion for the salivary component of SS. To assess its diagnostic effectiveness, we reviewed studies in which sialography was applied to patients with SS and control subjects for the purpose of estimating its diagnostic sensitivity and specificity or comparing it with other means of assessing salivary glands. Sialography appears to be diagnostically less sensitive but more specific than salivary flow rate measurement and more sensitive but less specific than labial salivary gland (LSG) biopsy. Such calculations are based on the diagnosis of SS established in each study, but the various studies used widely different criteria to establish that diagnosis. Therefore, these calculations are not based on a consistent standard, and comparison between the calculations may be misleading, which underscores the need to develop internationally accepted diagnostic criteria for SS. Studies conducted so far have not shown that parotid sialography is either a sensitive indicator of the salivary component of SS or more closely associated than LSG biopsy with keratoconjunctivitis sicca, the only other component of primary SS with which ultimately to assess diagnostic specificity.
9855836 Labial gland biopsy--a simple diagnostic procedure for Sjogren's syndrome. 1994 Dec Sjogren's syndrome is a collagen vascular disorder with multisystem. Its presentation may be similar to other diseases from which it can be differentiated by certain investigations--one of the most important being the labial salivary gland biopsy. A case report of Sjogren's syndrome is presented and the technique of the labial salivary gland biopsy is described.
8061138 [Pulmonary involvement in primary Sjogren syndrome]. 1994 May We present 6 cases of patients diagnosed of Primary Sjögren's Syndrome (PSS) according to Fox's criteria (1986). Our goal was to document the pulmonary affection with bronchoalveolar lavage, transbronchial biopsy and respiratory functional assessment. We verified the double affection described for PSS: interstitial and airways. In all the cases, independently of the respiratory radiological or functional findings, we observed a variable lymphocytary alveolitis. We did not find any correlationship between this alveolitis and the histological findings. We conclude that bronchoalveolar lavage and transbronchial biopsy in the PSS are useful for the detection of early pulmonary pathology.
8033645 [Adult recurrent sialadenitis of parotid gland and Sjogren's syndrome]. 1993 Nov This paper presents clinical, laboratory and sialographic studies on 22 patients with recurrent sialadenitis of parotid glands in adults (RSPA), 103 sjögren's syndrome (SS) cases, and particularly a long term (2 approximately 24 years, mean 10 years) follow-up studies on 22 patients with RSPA. Twelve out of 22 RSPA patients developed SS. These patients had recurrent parotid swelling for 1.5 approximately 14 years (mean 5.2 years) before xerostomia and keratoconjunctivitis sicca presented. Ten of the 22 RSPA patients did not develop SS, of them 10 experienced recurrent parotitis from childhood to adulthood, and the disease underwent remission in 7 of them. It is suggested to classify RSPA patients without any history of parotid swellings in childhood tentatively as subclinical SS (SCSS).
8495045 Systemic amyloidosis in a patient with adult onset Still's disease. 1993 Jan A 39-year-old woman presented clinical features of adult onset Still's disease. Seven years after the onset, she developed renal insufficiency and biopsy studies revealed amyloid deposits involving amyloid A protein, P component, lambda chain and kappa chain in the kidney and rectum. She died in 1992, primarily due to cardiac failure associated with amyloidosis, indicating that amyloidosis should be considered one of the fatal complications in adult onset Still's disease with a long history.
8327117 Asymptomatic cerebral involvement in Sjögren's syndrome: MRI findings of 15 cases. 1993 Fifteen patients with Sjögren's syndrome without clinical evidence of central nervous system disease were studied by MRI. Signal abnormalities were observed in 9 (60%). They were always visible on T2-weighted images as small punctate areas of high signal in the basal ganglia and the white matter of the cerebral hemispheres. Abnormalities were less frequently seen on T1-weighted images. Enlargement of cerebral sulci was observed in 6 cases. The specificity and significance of these abnormalities are discussed.
1496278 [Efficiency of cyclophosphamide bolus in Still's disease in adults. A case]. 1992 Apr The value of monthly intravenous injections of cyclophosphamide in adults with steroid-dependent Still's disease is documented by the report of a case. In the short term and perhaps also in the long term, this regimen reduces the amount of prednisone needed.
9023703 Overlapping cases with psoriasis and Sjögren syndrome: a study of lymphocyte response to 1996 Dec Sjgren syndrome (SjS) is an auto-immune disease and immunological mechanisms have recently been suggested in the pathogenesis of psoriasis; however, co-existence of these diseases is relatively rare. To determine whether or not there exist the nature of common underlying abnormalities, five patients with psoriasis and SjS were studied. Two patients had psoriasis vulgaris (PV), two had psoriasis arthropathica (PA), and the other had generalized pustular psoriasis (GPP). Lymphocyte response against staphylococcal enterotoxin B was examined by 3H-thymidine incorporation. In one patient with GPP, T-cell receptor (TCR) V beta repertoire of infiltrating T-cells into the lesional skin was examined by reverse transcriptase-polymerase chain reaction (RT-PCR). Stimulation index (S.I.) level of patients with psoriasis and SjS did not show any significant increases compared with patients with psoriasis or SjS alone; however, one patient with PV and SjS, who showed several serological immune imbalances, demonstrated a significant increase of S.I. level. Analysis of TCR V beta repertoire of the lesion of GPP showed strong expression of V beta 14 and 16 with mild expression of V 13-2 and 19, which may suggest that TCR V beta repertoire became oligoclonal in case of complication of these two disorders. In conclusion, our data did not suggest any common immunological responses to staphylococcal enterotoxin in induction of both psoriasis and SjS.
8712861 Assessment of the European classification criteria for Sjögren's syndrome in a series of 1996 Feb OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjögren's syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.
7774105 Fetal outcome in women with primary Sjögren's syndrome. A retrospective case-control stud 1995 Jan OBJECTIVE: To study fetal outcome in women with primary Sjögren's syndrome (SS) compared to that in women with systemic lupus erythematosus (SLE) and healthy women, and to study the possible association of fetal loss with anticardiolipin antibodies (aCL) and antibodies to SS-A/Ro and SS-B/La in women with primary SS. METHODS: A retrospective analysis of the fetal outcome in 55 pregnancies in 21 patients with primary SS compared to that in 100 pregnancies in 42 patients with SLE and 94 pregnancies in 42 healthy women matched for age, parity and the onset of the autoimmune disease with respect to pregnancy. IgG-, IgM- and IgA-aCL were determined by a cofactor-dependent ELISA and antibodies to SS-A/Ro and SS-B/La by ELISA using human recombinant antigens and affinity-purified antigens. RESULTS: Of all the 55 pregnancies in patients with primary SS, 8 (15%) occurred after the onset of primary SS symptoms. Eleven (20%) of the 55 pregnancies ended in fetal loss. The relative risk (RR) for fetal loss in patients with primary SS was 2.7 (95% CI 1.1-6.5; p = 0.023), and after the exclusion of the patient with four spontaneous abortions it was 2.0 (0.7-5.3; p = 0.18). In SLE the level of risk was 2.2 (0.9-5.0; p = 0.065). Fetal loss in patients with primary SS was not associated with elevated levels of anticardiolipin antibodies (aCL) or autoantibodies to SS-A/Ro or SS-B/La. Newborns of mothers with primary SS were not more premature or growth retarded than newborns of healthy women, but the absolute and the relative birth weights of the newborns of mothers with SLE was significantly lower than in healthy controls (P < 0.001 and P < 0.0001, respectively). CONCLUSION: We conclude that the majority of pregnancies in women with primary SS occur before the onset of the disease and that these women have an increased risk of fetal loss, which is not associated with elevated levels of ACL or antibodies to SS-A/Ro or SS-B/La. The risk of fetal loss in primary SS is similar to that in women with SLE, but fetal growth retardation appears to be more common in SLE than in primary SS.
7801198 [The clinical manifestations of Sjögren's syndrome in children]. 1994 Oct Sjögren's syndrome (SS) is thought to be uncommon in children. We studied the clinical manifestations and laboratory findings of 12 pediatric patients with SS, all of children did not have sicca symptoms but have lymphocytic infiltration of salivary glands, abnormal sialograms or abnormal results of scintigraphy compatible with typical SS. Seven cases had primary SS and five were secondary SS and had other autoimmune disorders (three cases with systemic lupus erythematosus, one case with dermatomyositis, and the other with mixed connective tissue disease). All patients were female. The mean age at onset of symptoms, including other autoimmune manifestations, was 12.2 years (range 9-15 years). The initial symptoms were some systemic manifestations (fever, exanthema, arthralgia, etc.) and various autoimmune phenomena (butterfly rash, Raynaud's phenomenon, proteinuria, weakness of muscles, etc.). On the other hand, no patients complained sicca symptoms. Laboratory studies in our patients revealed elevated levels of IgG (92%), antinuclear antibody (92%), rheumatoid factor (58%), anti-SS-A antibody (75%). These findings were similar to those found in adult patients with sicca symptoms previously reported in literature. From these studies, we suggest that lip biopsy, sialography and/or salivary gland's scintigraphy should be carried out in patients who had abnormal laboratory findings as mentioned above, irrespective of absence of sicca symptoms, in order to diagnose SS at early period.
7514111 Fibrinolysis-resistant fibrin deposits in minor labial salivary glands of patients with Sj 1994 May Minor labial salivary glands obtained at biopsy from 12 patients with Sjögren's syndrome were investigated by immunomorphological methods for the presence of fibrinolysis-resistant fibrin deposition. Fibrin could be found in extracellular localization between individual inflammatory cells infiltrating minor salivary glands. In the areas surrounding mononuclear infiltrations the labeling for fibrin showed an essentially fibrillar pattern. Staining for factor XIII A was observed over fibrin deposits and in large, stellate cells not showing reaction for fibrin. Here it is demonstrated that factor XIII A+ tissue macrophages are in an intimate relationship with fibrin deposits. The authors suggest that tissue macrophages may play a regulatory role in fibrin accumulation in association with autoimmune inflammation and consequently in demarcation of the inflamed tissue.
7939362 [What can be expected of the microscopic study of a biopsy of the accessory salivary gland 1994 Histological study is made on labial salivary gland biopsies in three usual circumstances: presence of a sicca syndrome; suspicion of a Gougerot-Sjogren's disease or of another systemic disease (sarcoïdosis; amyloïdosis). Its aim is: to appreciate the accuracy of the biopsy; to analyse elementary lesions and to suggest an etiologic diagnosis. The authors put emphasis on the interest: 1) to use new techniques of immunocytochemistry or molecular biology to appreciate the degree of evolutivity of lesions; 2) to quantify lymphoïd infiltrates, fibrosis and epithelial alterations; 3) to formulate results with regard not only to the etiological Diagnosis but also to the glandular Destruction by means of a double descriptive score: the "D.D." score.
8414039 Segmental anhidrosis in the spinal dermatomes in Sjögren's syndrome-associated neuropathy 1993 Sep We describe two women with primary Sjögren's syndrome and sensory neuropathy who had anhidrosis segmentally along the dermatomes of the spinal segment, along with sensory loss. Intradermal administration of cholinergic agents elicited no sweat response in the spinal segments with anhidrosis, whereas a normal response was present in the segments with obvious sweating. These features suggest segmental involvement of the postganglionic sympathetic ganglion cells.
8356981 Neuropsychiatric disease in Sjögren's syndrome: anti-ribosomal P and anti-neuronal antibo 1993 Aug PURPOSE: Patients with Sjögren's syndrome (SS) may develop nonfocal (i.e., psychiatric and/or cognitive dysfunction) as well as focal, neuropsychiatric disease (CNS-SS). Anti-ribosomal P and anti-neuronal antibodies have been associated with nonfocal neuropsychiatric disease in systemic lupus erythematosus (SLE), particularly psychosis and depression. This study examines the spectrum of psychiatric and cognitive dysfunction observed in SS patients with focal, as well as nonfocal, central nervous system (CNS) disease and relates these observations to the presence of serum and cerebrospinal fluid (CSF) anti-ribosomal and anti-neuronal antibodies. PATIENTS AND METHODS: One hundred thirty-one patients--patients with primary SS (n = 91), patients with secondary SS (n = 34), and mothers of infants with neonatal lupus erythematosus (NLE) (n = 6)--were studied. Patients were referred to a large tertiary referral center and the population was highly selected for CNS disease. Patients were evaluated clinically for focal and nonfocal CNS disease. Sera from 131 patients and 34 paired sera/CSF samples were examined by enzyme-linked immunosorbent assay and radioimmunoassay for the presence of anti-ribosomal P and anti-neuronal autoantibodies, respectively. Clinical features were categorized and autoantibody profiles obtained and correlated independently for statistical significance. Data were analyzed using the two-tailed Fisher exact test. RESULTS: Psychiatric or cognitive impairment, usually mild or moderate, occurred in over 80% (63 of 77) of this highly selected population of SS patients, and more than 60% of patients (48 of 77) had both. Anti-ribosomal P antibodies occurred in six (4.6%) patients with SS and related disorders. None of the patients with primary SS had anti-ribosomal P antibodies, whereas they were present in a small number of patients with secondary SS (i.e., 4 of 34 [12%]) and in 2 of 6 mothers of infants with NLE. There was no correlation between nonfocal CNS disease, including psychosis or severe depression, and the presence of anti-ribosomal P antibodies. Paired serum CSF samples from 34 SS patients with active CNS disease, including 6 with psychosis and 5 with severe depression, did not contain either anti-ribosomal P or anti-neuronal antibodies. Anti-ribosomal P and anti-neuronal antibodies were present in a control subset of SLE patients defined serologically by the presence of anti-nDNA antibodies. CONCLUSION: Patients with primary SS associated with CNS disease, including psychosis and depression, do not have serum or CSF autoantibodies to ribosomal P peptide or neuronal antigens, detected by binding to neuroblastoma cells. Thus, autoantibodies associated with nonfocal or diffuse CNS disease in classical SLE (particularly psychosis and depression) are not present in CNS-SS. The observations suggest that nonfocal CNS disease in CNS-SS and CNS-SLE may be mediated by different immunopathologic mechanisms. Potentially, these observations may have diagnostic and therapeutic implications in the management of patients with CNS-SS and patients with CNS-SLE.
7689780 Correlation between impression cytology and tear function parameters in Sjögren's syndrom 1993 Jun We have compared clinical (Schirmer I test, BUT and rose bengal staining), laboratory (lysozyme and lactoferrin tear levels) and histological tests (impression cytology) in 165 eyes from 85 patients with primary Sjögren's syndrome and in 80 eyes from 40 control subjects. Impression cytology can provide the location of cellular alterations on the ocular surface and information on the severity of the disease. The upper bulbar and interpalpebral areas from patients with primary Sjögren's syndrome were shown to have cellular alterations early in the course of the disease, while the lower bulbar and lower palpebral areas were only affected in severe cases. Statistical analysis has shown that impression cytology and rose bengal staining are the most specific and sensitive methods for the diagnosis of primary Sjögren's syndrome.
8201927 [Spontaneous splenic hypofunction in systemic lupus erythematosus and primary Sjögren syn 1993 The objective of this study was to evaluate hyposplenism in autoimmune diseases by the presence of Howell-Jolly bodies in blood erythrocytes and 99Tc spleen scan. Blood smears of 174 patients with autoimmune diseases and 126 controls were studied. Other possible causes for the presence of Howell-Jolly bodies were excluded. Evidence of hyposplenism was demonstrated in 4 of 79 patients with Systemic Lupus Erythematosus (SLE) and in 2 of 18 cases of primary Sjögren Syndrome (PSS), whereas no hyposplenism was found in the remaining cases of other autoimmune diseases. In one of the patients with SLE, hyposplenism was transient. Among the control cases, a patient with Chronic Lymphocytic Leukemia with splenomegaly presented hyposplenism. IN CONCLUSION: 1) Hyposplenism is more frequently found in SLE and PSS than in other autoimmune diseases. 2) Hyposplenism in autoimmune diseases can be transient. 3) Splenomegaly and enlarged spleen scan do not exclude hyposplenism.
1432358 Chemically humanized murine monoclonal antibody against a cell nuclear antigen: usefulness 1992 The cellular nuclear antigen SS-B/La is known to be a major antigenic target to an autoantibody in patients with Sjogren's syndrome and systemic lupus erythematosus. It is useful to detect an anti-SS-B/La antibody from patients' sera in a clinical point of view. We purified SS-B/La from rabbit thymus acetone powder by affinity chromatography with a murine anti-SS-B/La monoclonal antibody (1C3-H7). An enzyme-linked immunosorbent assay method, in which SS-B/La was used to coat a plate, was also successfully established. It is difficult to obtain a large volume of patient's serum with high antibody titer and high specificity as a positive control. We investigated whether or not a positive control from human could be replaced by a murine monoclonal antibody to SS-B/La. The 1C3-H7 was conjugated with a human IgG Fc' fragment using N-gamma-maleimidobutyryloxysuccinimide as a cross-linker. The chemically humanized murine monoclonal antibody (1C3-Fc') was recognized by antiserum specific for human IgG Fc fragment. 1C3-Fc' reacted to SS-B/La but not to other antigens. Furthermore, the titration curve of this conjugate ran parallel with those of patients' sera specific for SS-B/La. It is concluded that a chemically humanized murine monoclonal antibody is useful as a positive control in place of a human patient's serum.