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ID PMID Title PublicationDate abstract
8323383 Hydroxychloroquine treatment for primary Sjögren's syndrome: a two year double blind cros 1993 May OBJECTIVES: In 1985 and 1988 a positive effect of treatment of primary Sjögren's syndrome with hydroxychloroquine was reported in two small open studies. To investigate further the clinical and laboratory effects of hydroxychloroquine in primary Sjögren's syndrome a two year study was performed. METHODS: The design of the study included a prospective, placebo controlled, two year double blind crossover trial in 19 patients. RESULTS: A significant decrease in IgG and IgM and a tendency for a decrease in the erythrocyte sedimentation rate (ESR) during treatment with hydroxychloroquine compared with treatment with placebo were found. No beneficial clinical effect of the use of hydroxychloroquine as expressed in preference for treatment with hydroxychloroquine or placebo with regard to symptoms and signs of primary Sjögren's syndrome could be shown, however, nor any relevant change in tear gland activity and sequelae of peripheral tear function deficiency, nor salivary gland scintigraphy. CONCLUSIONS: The use of hydroxychloroquine at a dose of 400 mg daily taken over a 12 month period does not have a worthwhile clinical benefit in patients with primary Sjögren's syndrome despite an improvement of hyperglobulinaemia and slight changes in the ESR and IgM.
7481583 Antibodies against oxidised low-density lipoprotein in juvenile chronic arthritis. 1995 Enhanced lipid peroxidation, reported to take place in rheumatoid joints and suggested to play a significant role in joint inflammation, led us to study the occurrence of antibodies against oxidised low-density lipoprotein (Ox-LDL Ab) in patients with juvenile chronic arthritis. Enzyme-linked immunosorbent assay was used to detect Ox-LDL Ab and antiphospholipid antibodies (aPL Ab) in sera from 84 patients and 91 controls. Elevated levels of Ox-LDL Ab were found in 14 patients (17%) as opposed to 4 controls (4%; p < 0.01). Similarly, 14 patients had an elevated aPL Ab level and a fairly good correlation between Ox-LDL Ab and aPL Ab (r = 0.52) existed in the patients. The increased frequency of elevated levels of Ox-LDL Ab may reflect lipid peroxidation occurring in rheumatoid joints but crossreactivity with aPL Ab for the induction of Ox-LDL Ab cannot be excluded.
9005668 [The normal tear fluid and decreased tearing in patients with Sjögren's disease and Sjög 1996 Mar Studies of total lipids--squalene, cholesterol and sterol ethers, fatty acids and their ethers, lanosterol, oxysterols and phospholipids in 25 patients (50 eyes) with Sjogren's disease and 26 patients (52 eyes) with Sjogren's syndrome have revealed a statistically significant reduction of phospholipid quantities and an increase in fatty acids, and cholesterol in patients vs. the normal subjects. Possible influence of lipid components on the structure and function of the tear pellicle is discussed.
8838512 Biopsy of labial salivary glands and lacrimal glands in the diagnosis of Sjögren's syndro 1996 Jan OBJECTIVE: To determine the diagnostic value in Sjögren's syndrome (SS) of evaluating the presence of focal adenitis in labial salivary gland (LSG) and lacrimal gland (LG) biopsy specimens. METHODS: We studied 105 cases with suspected SS and performed biopsies prospectively. The diagnostic criteria of Fox, et al were followed. The Chisholm-Mason grading standard and Greenspan focus score were used for evaluation. RESULTS: Epimyoepithelial islands and severe lymphocyte infiltration with germinal centers were observed only in LG biopsy specimens (5.9 and 3.5%, respectively). The lymphocytic focus scores of LG (2.5 +/- 2.1/4 mm2) were significantly higher than those of LSG specimens (1.3 +/- 1.1/4 mm2) in 23 cases with both biopsy results (p < 0.05). Six of the 32 LSG biopsies were normal while their LG biopsies were significantly higher. Conversely, 5 of the 32 LG biopsies were aided by corresponding LSG biopsies, since in LG biopsies no glandular tissue was obtained. Evaluation of both specimens for the same patient was significantly more effective in diagnosing SS than using either specimen alone (p < 0.01). CONCLUSION: LG biopsy specimens have a more evident histopathology than LSG biopsy specimens. We recommend that both LSG and LG biopsies be performed in patients with suspected SS to reduce false negative results and improve diagnostic accuracy.
7555272 Sialographic follow-up study of patients with Sjogren's syndrome. 1995 Jul Sialographic follow-up studies of 36 cases of Sjogren's syndrome (SS) were performed with the duration of 5-72 months (mean 25 months). Three groups were divided according to the international criteria: secondary group, 14 cases (xerophthalmia, or/and xerostomia, and connective tissue diseases), primary group, 14 cases (xerostomia and xerophthalmia), and possible group, 8 cases (xerophthalmia or xerosptomia only). Sialographic findings at follow-up examination included (1) delayed evacuation, which may occur as early as 5 months later; (2) increased punctate sialectasis; (3) decreased punctate sialectasis with globular increase; (4) punctate, globular, and cavity sialectasis with radiolucent sialolith; and (5) centripetal atrophy. The cases with secondary and possible SS without xerostomia demonstrated little involvement of the parotid gland, without sialectasis. But sialodochitis was shown when radiolucent sialolith formed. The stimulated whole salivary flow rate (SWSFR) was closely related to sialographic appearance, i.e., the cases with lower value of SWSFR showed more siatectasis sialographically, but with higher SWSFR value, and less sialectasis. The relationship between the SS and pyogenic or candidal infection was discussed.
7772528 Factor V inhibitor associated with Sjögren's syndrome. 1995 Apr We report an unusual case of a 74-year-old male who developed a serum autoantibody reactive with human coagulation factor V (FV) in an activated form, as demonstrated by coagulation studies and immunoblotting analysis. Despite marked prolongation of a prothrombin time and an activated partial thromboplastin time in this patient, the inhibitor was not associated with clinical bleeding but with multiple cerebral infarctions. The patient had suffered from Sjögren's syndrome with polyclonal hypergammaglobulinaemia. The patient's purified IgG, an immediately acting inhibitor to FV, reacted with a light chain of thrombin-activated FV (FVa) and inhibited the procoagulant activity of FVa without affecting the cleavage of FVa by activated protein C. The FV inhibitor may arise from activation of FV with consequent exposure of neoantigen during the activation of coagulation cascade in the patient with an autoimmune disorder for the background.
7553055 [A case of adult onset Still's disease complicated with adult respiratory distress syndrom 1995 Apr A 71 year-old man with adult onset Still's disease was admitted to our hospital because of fever, sore throat, myalgia and macular nonpruritic salmon pink eruption. He was treated with prednisolone, 40 mg daily and these symptoms disappeared. When the dose of prednisolone was reduced to 30 mg daily, he began to notice fever. 5 days later he developed adult respiratory distress syndrome (ARDS). The dose of prednisolone was increased to 50 mg daily and oxygen administration was started. All symptoms began to improve immediately and the dose of prednisolone was decreased to 40 mg daily. 10 days later he noticed fever and skin rash. Laboratory investigation showed platelet counts of 69,000/mm3, a ferritin of 37,000 ng/ml, and increased fibrinogen degradation product, indicating increased activity of adult onset Still's disease associated with disseminated intravascular coagulation (DIC). The dose of prednisolone was again increased to 60 mg daily, and 100 mg of nafamostat mesilate was administrated intravenously. All above symptoms associated with adult onset Still's disease and DIC disappeared. The dose of prednisolone was gradually decreased and the clinical course was uneventful with daily administration of 10 mg of prednisolone. Although there are a couple of case report which described the association of adult onset Still's disease with either ARDS or DIC, the association of adult onset Still's disease with both ARDS and DIC have not been reported yet.
7731118 [Progressive pulmonary fibrosis in a case of Sjögren's syndrome with asbestos exposure]. 1995 Feb A 62-year-old man had an occupational history of exposure to asbestos for 27 years (1947-1974). Dry mouth, dry eyes and swollen gums were noted in 1974. Sjögren's syndrome was diagnosed in 1975. The chest radiograph in 1984 showed bilateral pleural thickening and small reticular opacities in the left lower lung field. In 1990, he complained of dyspnea and cough, and diffuse interstitial pneumonia was diagnosed. While being treated for diffuse interstitial pneumonia, pulmonary fibrosis progressed rapidly, and advanced pulmonary fibrosis was obvious in 1992. Laboratory data showed hyper gamma-globulinemia (36.8%) and a high level of IgG (3772 mg/dl) in 1976. These values decreased to within the normal ranges during the subsequent clinical course. The results of lymphocyte subset counts in 1988 were normal. With the progression of diffuse interstitial pneumonia in 1990, the lymphocyte subset counts showed a low percentage of CD4 (19.2%), a low CD4/8 ratio (0.46), and a low percentage of CD20 (26.8%). In 1992, as pulmonary fibrosis progressed despite treatment, the disorder of lymphocyte subsets became worse. The CD4 percentage was very low (5.0%), as was the CD4/8 ratio (0.08), and the percentage of CD20 (1.4%); the CD8 percentage was high (64.7%), as was the percentage of Leu 7 (49.0%). These immunologic and pulmonary changes could be not explained by Sjögren's syndrome. Determining what factor induced these changes is difficult, but asbestos exposure is a likely cause.
1609242 [Treatment of Still's disease with bolus methylprednisolone. Retrospective study of 7 pati 1992 Mar The problems raised by the treatment of Still's disease in the child or adult led the authors to evaluate the short term clinical efficacy of bolus doses of methylprednisolone. Seven patients were given 12 boluses, followed by oral corticosteroids at 5 to 10 mg in 9 cases out of 12. Still's disease was active in all cases. A frank clinical improvement was seen on the fourth day. The response duration was 3 weeks to 20 months, with a mean follow-up period of 7 months. There were 3 early relapses, 5 late relapses between 2 and 20 months and 4 patients are still in remission at 5, 6, 15 and 19 months. There were no immediate adverse reactions. These results suggest the usefulness of boluses of methylprednisolone in exacerbations of Still's disease, avoiding the disadvantages of high dosage daily corticosteroids.
8959535 Cerebral haemorrhage complicating adult-onset Still's disease: a case report. 1996 Nov The case of a 75-year-old Japanese woman with adult-onset Still's disease who presented with cerebral haemorrhage is described. She had been in clinical remission for 2 years, after induction therapy including non-steroidal anti-inflammatory drugs, prednisolone, cyclophosphamide and mizoribine followed by auranofin, until her cerebral haemorrhage occurred, although her serum level of ferritin had gradually increased. After the onset of cerebral haemorrhage, the patient's serum level of thrombomodulin was elevated although c-reactive protein and lactate dehydrogenase were not increased. Anti-cardiolipin antibody and lupus anti-coagulant were not detected. Patients with adult-onset Still's disease are rarely reported to develop cerebral vascular disease, possibly because the disease is most frequent in young adults. The cerebral haemorrhage may have been caused by the vasculitis due to Still's disease.
7789054 The diagnosis value of beta 2-microglobulin and immunoglobulins in primary Sjögren's synd 1995 Mar Salivary and serum concentrations of beta 2-microglobulin, salivary levels of the immunoglobulins, and salivary flow (as measured by a gravimetric method) were assessed in 34 patients and 11 controls. Of the 34 study patients, 16 had a primary form of Sjögren's syndrome (pSS) and 18 had sicca syndrome. The salivary and serum concentrations of beta 2-microglobulin and salivary levels of IgA and IgG were much higher in the patients with pSS than in controls or those with sicca syndrome (p < 0.001). Similarly, the salivary IgM levels of patients with pSS were higher (although at a lower degree of statistical significance) as compared to those of patients with sicca syndrome (p < 0.01) or controls (p < 0.05). No correlation was observed among the salivary flow and the salivary IgG and IgM levels, while there was a negative relationship (p < 0.01 with salivary IgA levels in all 45 patients considered as a whole. In 12 patients with pSS, the coefficient of correlation between the salivary gland biopsy focus score and the salivary (but not serum) concentration of beta 2-microglobulin was highly significant (p < 0.001). A similar relationship was observed with the IgG (p < 0.001) and IgM (p < 0.05) levels, but not IgA. In the diagnosis of pSS, the salivary IgA level demonstrates high sensitivity and low specificity. In contrast, serum and salivary concentrations of beta 2-microglobulin, salivary IgG, and to a lesser degree, salivary IgM have high specificity and positive predictive value.(ABSTRACT TRUNCATED AT 250 WORDS)
7767334 Primary systemic Sjögren syndrome (SS) preceding systemic lupus erythematosus: a retrospe 1995 Feb That SS and SLE can coexist is well established. The time relationship between the two diseases is however not well known. To determine whether SS can precede SLE, we reviewed the charts of 55 patients with SS diagnosed in our department between 1975 and 1985. All 55 patients fulfilled at least four of the six preliminary criteria for SS developed by the European Study Group for SS. At entry, none met four ARA criteria for SLE and none had anti-double-stranded DNA or anti-Sm antibodies. The mean follow-up was 12 years (range 8-18 years). Four of the 55 patients developed SLE (7.5%). High titres of anti-native DNA antibodies (> 35 IU RIA, normal < 7 IU) appeared in four patients (2-11 years after the diagnosis of SS). Two of these patients fulfilled at least four ARA criteria for SLE; two others had only three criteria. Two additional patients developed anti-Sm antibodies without anti-native DNA antibodies 1 and 10 years after the initial diagnosis of SS, respectively. Both patients fulfilled six ARA criteria for SLE. At entry, anti-SSA and/or anti-SSB antibodies were present in all six SS patients and persisted subsequently. The main clinical events suggesting progression to SLE were pleuropericarditis, glomerulonephritis and focal central nervous system disease. In conclusion, SS with extraglandular components can antedate SLE by many years. Anti-native-DNA and anti-Sm antibodies should be measured when atypical clinical manifestations occur in a patient with systemic SS.
7653945 [Association of Gougerot-Sjögren syndrome and viral hepatitis C. Apropos of 6 cases]. 1995 Hepatitis C virus (HCV) has been found in the serum of 3 out of 109 patients with Sjögren's syndrome, whereas Sjögren's syndrome has been demonstrated in 3 out of 7 HCV-positive patients. The six HCV-positive Sjögren's syndrome patients were compared with 95 HCV-negative Sjögren's syndrome patients. Anti-smooth muscle antibodies were detected in 1 of the former group and 6 of the latter. Cryoglobulin existed in 1 and 6, respectively. The levels of circulating IgA and IgA-containing immune complexes were lower in the HCV-positive than in the HCV-negative patients.
8778989 Clinicopathological factors relating malignant lymphoma with Sjögren's syndrome. 1996 Apr This study was undertaken to summarize the current status of the clinicopathologic factors related to non-Hodgkin's lymphoma (NHL) development in patients with Sjögren's syndrome (SS). Through a MEDLINE search, pertinent articles on SS, monoclonality, and NHL were found and reviewed. Malignant lymphoma description was based on the International Lymphoma Study Group classification. Patients with SS are known to have an increased risk for developing B-cell NHL (B-NHL). However, such a complication occurs in less than 10% of patients, being reported mainly in those with primary SS. Extranodal low-grade B-NHL are observed most frequently. Persistent enlargement of parotid glands, adenopathy, monoclonal gammopathy, and cross-reactive idiotypes are all signs suggesting possible lymphoma evolution. Although monoclonality does not mean malignancy unequivocally, it is considered to be a precursor for NHL development in SS. Factors implicated into lymphomagenesis in SS include dysregulation in the mechanisms leading to apoptosis, hyperstimulation of B-1 cells, and an infectious agent. Polyclonal lymphoproliferation characterizing SS might in some instances transform into monoclonal, and then to malignancy. Further studies on the mechanism whereby NHL develops in SS are warranted.
7878130 Taste performance in Sjogren's syndrome. 1995 Jan Sjogren's Syndrome (SS) patients have impaired salivary gland function and an elevated frequency of oral complaints. The taste complaints are thought to be due to sensory deficits that arise in the absence of sufficient saliva to maintain taste receptors. We assessed the subjective complaints, salivary production and taste functioning of SS patients and unaffected individuals. We found the expected decreased salivary gland function and increased frequency of taste complaints. Our taste assessment with weak stimuli confirmed and expanded the previous report of decreased taste threshold sensitivity in SS. However, perception of stronger taste stimuli was not impaired. In contrast with previous reports, patient judgments of intensity were not significantly reduced for any of the four taste qualities. Although the salivary gland function of all patients was markedly impaired relative to that of controls, patients lacking measurable salivary flow were no more likely than patients with residual function to exhibit subjective complaints or taste impairments. Our observations are inconsistent with a simple causal chain running from salivary gland dysfunction to sensory loss to taste complaints.
8016580 Sjögren's syndrome in inflammatory rheumatic diseases: analysis of the leukocyte protein 1994 In a hospital population of 154 patients with a wide range of inflammatory rheumatic diseases, patients with sicca symptoms were subjected to objective ocular and oral tests to establish cases with Sjögren's syndrome (SS). The plasma level of the leukocyte protein calprotectin has been shown to be a good indicator of disease activity and inflammation in various rheumatic diseases. In the present study, calprotectin levels in plasma and whole saliva were analysed and evaluated as potential markers of SS and salivary gland disease activity. Plasma calprotectin levels did not differ significantly between patients with SS and patients with no sicca symptoms. However, salivary calprotectin levels correlated significantly with the plasma calprotectin levels and with several ocular variables, weakly with salivary flow and serum rheumatoid factor, but not with focal sialadenitis. In conclusion, this study shows that salivary calprotectin levels seem to be associated with several variables of SS glandular pathology, indicating the need for further and more comprehensive studies on calprotectin in various oral fluids and in lacrimal fluid in relation to SS glandular disease activity.
10412476 Effects on local blood flux of acupuncture stimulation used to treat xerostomia in patient 1993 Sep Twenty-one patients with Sjögren's syndrome were given four different kinds of acupuncture stimulation, at acupuncture points previously used to treat xerostomia. The local blood flux in the skin overlying the parotid gland was measured with laser Doppler flowmetry before, during and after the acupuncture stimulation. The results showed that the local blood flux increased significantly during and after both manual acupuncture and low-frequency (2 Hz) electro-acupuncture as compared with superficial acupuncture. These results indicate that acupuncture induced an increase in the local blood flux which was more pronounced for those patients who had previously reacted with increased salivary flow to acupuncture.
8832990 Comparative study of 6 types of criteria in adult Still's disease. 1996 Mar OBJECTIVE: To compare the sensitivity of 6 sets of criteria proposed for adult Still's disease in published papers in a well defined patient population. METHODS: For inclusion we required a diagnosis of adult Still's disease established by a senior internist or senior rheumatologist. Sensitivity was evaluated in 65 new cases of adult Still's disease from a multicenter study based on signs and symptoms (1) in the first 6 months of illness, (2) in the same period in patients followed more than one year (52 patients), and (3) at any time in patients followed more than one year. RESULTS: The Yamaguchi criteria are most sensitive (93.5%), followed by those of Calabro and Londino. Medsger et al (80.6%), Kahn (69.3%), Reginato (55.2%), and Goldman (43.7%). Sensitivity was high even from the first 6 months, except for the 2 less sensitive criteria. CONCLUSION: Awareness of these discrepancies in adult Still's disease criteria are crucial to ensure comparability of different series of this condition.
8161735 A case of myelodysplastic syndrome progressing to acute myelocytic leukemia in which adult 1993 Dec A patient with adult-onset Still's disease who presented with myelodysplastic syndrome (MDS) after a course of 6 years is reported. To our knowledge, this is the first such reported case. The patient died of acute myelocytic leukemia. The possibility that cyclosporine contributed to the onset of MDS is discussed.
1296353 [Adult-onset Still's disease--the effect of methylprednisolone pulse therapy]. 1992 Dec The authors describe in a 40-year-old woman Still's disease with an adult onset. The activity of the disease was not controlled by large Prednisone doses and therefore pulsed treatment with methylprednisolone with very favourable results was used. The authors discuss the use of pulsed treatment with methylprednisolone in this disease.