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ID PMID Title PublicationDate abstract
8531353 [Expression of ductal Fas antigen in sialoadenitis of Sjögren's syndrome]. 1995 Oct The expression of Fas antigen on ductal epithelial cells of sialoadenitis was examined in patient with Sjögren's syndrome and normal subject. In two patients with severe sialoadenitis, Fas antigen was strongly expressed on the ductal epithelial cells. In contrast, Fas antigen was not seen in minor salivary gland of normal subjects and of mild sialoadenitis cases. This finding suggests that Fas antigen may play a role in the pathogenesis of sialoadenitis in Sjögren's syndrome by providing a specific target for cytotoxic T cells expressing Fas ligand.
7746963 [Treatment of Still disease in adults with intravenous immunoglobulins]. 1995 In most cases, the treatment of adult's Still disease presents difficulties, in view of its undesirable side-effects. For this reason, we made an open trial of the effects of high-dose intravenous immunoglobulins (IVIg), a therapy with low iatrogen risk, and whose effectiveness in the treatment of other multisystemic diseases has been acknowledged. Seven patients suffering from adult Still's disease were given between one and eight IVIg infusions with a dose of 1 g/kg/day for two consecutive days. All seven responded positively, with clinical improvement. It lasted between 1 and 90 days in three patients, who subsequently relapsed; it has been continuing for an average of 13 months (2 to 24 months) in the other four patients. There were no clinical features making it possible to distinguish, after the trial, the patients who responded positively to intravenous immunoglobulins. This data, which needs to be confirmed with a controlled trial, provides hope of improved therapy for the one half of patients suffering from adult Still's disease who respond positively to intravenous immunoglobulins.
8029627 [Biopsy of the accessory salivary glands. 5 years' experience]. 1994 Biopsy of the minor salivary glands has become a routine examination ordered by physicians working in a wide range of disciplines in order to search for or eliminate the diagnosis of Gougerot Sjögren's disease or another systemic disease. We emphasize the need to use this examinations as a part of a complete work-up of the buccal cavity and the salivary glands. We reviewed our experience with 1,500 biopsies. The glands biopsied were normal in 56% of the cases and led to the diagnosis of Gougerot Sjögren's disease in 24%, chronic sialadenitis in 10% and diverse trophic problems in 5%. The clinical stage of Gougerot Sjögren's disease is usually proposed according to the Chisholm classification which we propose to compare with the Chomette classification. Finally, we described the technique of minor salivary gland biopsy.
7819797 [Primitive Sjögren's syndrome in France and Ecuador. Possible involvement od HTLV 1]. 1993 The authors study the possible involvement of retrovirus in Sjögren's syndrome. They report a preliminary study of 20 French and 13 Ecuadorian patients with a primary Sjögren syndrome. A previous epidemiologic study showed a HTLV1 seroprevalence going from 0.04 to 6% on different regions of Ecuador. The results of this study show the presence of anti-HTLV1 antibodies only in 2 Ecuadorian patients and does not allow a final conclusion of the role of HTLV1 virus. Nevertheless other studies continue on the serie using minor salivary gland specimens obtained by biopsy. Recent publications and the notion of an "endogen" retrovirus will may be lighten a new day in this research.
7706337 Osteolysis around cementless porous-coated anatomic knee prostheses. 1995 Mar We report the incidence of osteolysis in the femur, tibia, and patella of 44 consecutive patients (60 knees) who were followed for more than seven years after cementless knee arthroplasty with a Porous-Coated Anatomic prosthesis. The average age of the patients was 56.5 years (17 to 73); the operative diagnosis was osteoarthritis (33 knees), rheumatoid arthritis (17), tuberculous arthritis (7) and post-traumatic arthritis (3). All patellae were resurfaced. No femoral or tibial component was loose at the final follow-up examination. Thirty patellar components were loose of which six had been revised. Radiographs revealed osteolysis in 90% of the tibial plateaux and in 80% of the 30 intact patellar prostheses. No osteolysis was seen around any femoral component. In 50 knees (83%) the average wear of the polyethylene liner was 2.5 mm in the medial compartment and 1.7 mm in the lateral compartment. Four of 60 knees (6.7%) were revised for complete wear of the polyethylene liner of the tibial component. Fixation of the tibial and patellar components without cement fails to seal the interface between bone and prosthesis and allows the migration of polyethylene particulate debris which causes osteolysis.
8921964 Autoimmune disease of exocrine organs in immunodeficient alymphoplasia mice: a spontaneous 1996 Nov Mice homozygous for an autosomal recessive mutation aly (alymphoplasia) lack both lymph nodes and Peyer's patches, and show defects in both humoral and cellular immunity. Histopathological analysis revealed chronic inflammatory changes in exocrine organs such as the salivary gland, lacrimal gland, and pancreas of the homozygotes (aly/aly), but not the heterozygotes (aly/+). In these exocrine organs, mononuclear cells consisting mainly of CD4+ T cells infiltrate periductal areas, and, in some cases, the cell infiltration extended to lobules. The inflammatory changes in exocrine organs were transferred by a T cell-enriched fraction of spleen cells from homozygous animals. These results suggest that autoimmune mechanisms mediated by self-reactive T cells may be involved in the inflammatory lesions of various exocrine organs in the homozygous mice, although these mice show immunodeficiency. Inflammatory changes were also observed in the lung of the homozygotes. Since Sjögren's syndrome is characterized by diffuse lymphocyte infiltration in the periductal areas of the lacrimal and salivary glands and is occasionally associated with pulmonary disease, aly/aly mice may serve as a unique spontaneous model of Sjögren's syndrome.
8933193 Unilateral acute exacerbation of pulmonary fibrosis in association with Sjögren's syndrom 1996 Oct A 70-year-old female had an abnormal chest roentgenogram. Infiltrative shadows were recognized in the right lung, and an open lung biopsy (OLB) specimen revealed usual interstitial pneumonia (UIP). Xerostomia, keratoconjunctivitis sicca, and lymphocyte infiltration in salivary glands were consistent with Sjögren's syndrome; she was diagnosed as having pulmonary fibrosis in association with Sjögren's syndrome (SjS-IP). Acute exacerbation occurred and she was successfully treated with corticosteroids. Unilateral exacerbation and UIP in SjS-IP are rare. OLB is useful for diagnosis and to select a pertinent therapy if lung involvement is unilateral in Sjögren's syndrome.
8828331 [A case report of coronary artery bypass grafting in a patient with Sjögren's syndrome]. 1996 Sep There have been very few reports on coronary artery bypass grafting (CABG) during collagen disease. This paper reports a 49-year-old female suffering angina after myocardial infarction who revealed preoperatively findings such as arthralgia, thrombocytopenia and positive anti-nuclear antibody. She underwent CABG and was definitely diagnosed as Sjögren's syndrome after the surgery. The coronary artery lesion in this case was mainly attributed to coronary arteritis due to Sjögren's syndrome because she was comparatively young and no other coronary artery risk factors such as hypertension, hyperlipidemia, thickened intima or angitis had been found by aortic pathology. Moreover, anticardiolipin antibody, which is highly related to the myocardial infarction in juveniles, was positive in this case.
8639175 Aberrant expression pattern of the SS-B/La antigen in the labial salivary glands of patien 1996 May OBJECTIVE: Salivary glands of patients with Sjögren's syndrome (SS) have been shown to be a site of anti-SS-B/La antibody production. The present study investigated differences in the localization of the SS-B/La antigen in labial salivary gland (LSG) tissue between SS and non-SS patients, which may explain the local antigen-driven anti-SS-B/La response. METHODS: Distribution of SS-B/La was studied immunohistologically in the LSG biopsy samples of 9 SS patients, 10 non-SS patients, and in normal tissues obtained at autopsy within 2 hours after death, using a mouse monoclonal antibody directed to SS-B/La. In 3 SS and 3 non-SS patients, LSGs were also studied with affinity-purified biotinylated human antibodies directed against SS-B/La. RESULTS: In the non-SS patients, SS-B/La was primarily observed in the nucleoli of acinic cells of the LSGs. Patients with either primary SS or secondary SS showed an accumulation of SS-B/La in the nucleoplasm of acinic cells. In the SS patients, SS-B/La was also detected in the cytoplasm as a diffuse or perinuclear staining. Sometimes, SS-B/La was found along the membrane of acinic cells as well. This aberrant nuclear and cytoplasmic distribution of SS-B/La in SS patients correlated well with abnormalities in the composition of the plasma cell population in the LSGs, but not with a lymphocytic focus score > 1. CONCLUSION: The accumulation and redistribution of SS-B/La in the LSGs may play an important role in the local antigen-driven anti-SS-B/La response in SS, and can also be used to improve the diagnostic possibilities of the LSG biopsy.
8624149 Similar Ro/SS-A autoantibody epitope and titer responses in annular erythema of Sjögren's 1996 May BACKGROUND AND DESIGN: Studies were conducted to determine whether certain types of Ro autoantibodies are unique to subacute cutaneous lupus erythematosus and annular erythema of Sjögren's syndrome. Ten American subacute cutaneous lupus erythematosus sera, 11 Japanese annular erythema of Sjögren's syndrome sera, and 39 control sera were tested by enzyme-linked immunosorbent assay with recombinant 52- and 60-kd Ro fusion proteins, native 60-kd Ro protein, and native La/SS-B protein. RESULTS: Japanese annular erythema of Sjögren's syndrome sera and American subacute cutaneous lupus erythematosus sera share several types of anti-52-kd and anti-60-kd Ro autoantibodies. However, these antibodies were found significantly more often and in significantly higher titers in sera from patients with Sjögren's syndrome who did not manifest these skin diseases. Patients with high Ro autoantibody titers were likely to have overt exocrine gland dysfunction. CONCLUSIONS: The failure to identify disease-specific Ro autoantibodies in subacute cutaneous lupus erythematosus and annular erythema of Sjögren's syndrome sera suggests that additional factors influence the development of these skin diseases. However, similarities in the Ro autoantibody responses and in the clinical features of subacute cutaneous lupus erythematosus and annular erythema of Sjögren's syndrome suggest that these two skin diseases might arise from a similar pathogenic process.
8531359 [Possible involvement of Epstein-Barr virus in the pathogenesis of Sjögren's syndrome]. 1995 Oct Research into the role of Epstein-Barr virus (EBV) in the pathogenesis of SS has been a focus of interest for the past decade. The use of EBV as a probe for cellular and humoral immune responses has contributed to our current understanding of SS. However, it is still difficult to assign a role to EBV in the pathogenesis of SS. We have already demonstrated a) increased excretion of EBV in the saliva of SS, increased levels of EBV DNA in salivary gland biopsies of SS patients and spontaneous and massive production of transforming EBV in B cell lines established from SS patients. These data suggest that the reactivation of EBV might be deeply involved in disease perpetuation, polyclonal B cell activation and B cell malignancy in SS, even if it is not the primary cause. Recently, we examined the nucleotide sequence of the U2 region in EBV obtained from SS patients. The U2 region contains genes encoding EBNA-2, which plays an important role in B cell transformation and activation. In addition, studies on the breakdown of self-tolerance, and intriguing evidence supporting a potential role for infectious agents such as EBV and retroviruses also offer novel views of inflammation of salivary gland. This review will discuss recent advances in these subjects.
7653942 Participation of cryoglobulinaemia in the severe peripheral neuropathies of primary Sjögr 1995 A peripheral neuropathy is encountered in 10 to 50% of patients with primary Sjögren's syndrome (pSS). The pathogenesis of this complication remains unknown, but severe cases of peripheral neuropathies seem to result from a vasculitis. Since about 20% of pSS are associated with a cryoglobulinaemia, we initiated a retrospective study to assess the participation of cryoglobulinaemia in the severe peripheral neuropathies (SPN) of pSS. We found 6 cases of SPN (presence of pain and/or muscle weakness) in a group of 115 pSS (5%): 3 polyneuropathies and 3 mononeuritis multiplex. In all cases, the involvement was sensoromotor and axonal. Four patients had a biopsy-documented cutaneous vasculitis, and 2 of them had also a muscular lymphocytic vasculitis. A cryoglobulinaemia was detected in sera of 5 patients. Although the research was negative in the remaining patient, the presence of an IgM monoclonal gammapathy and of an hypo-complementaemia suggested also the presence of a cryoglobulinaemia. Complement was activated in the 6 cases. Treatment was aggressive: oral corticosteroid (6 cases), methylprednisolone pulses (4 cases), chloraminophen (1 case), plasma exchanges (2 cases), intravenous immunoglobulins (1 case). A stabilization or a partial regression of the neuropathies were observed in 1 case and 5 cases, respectively. This was associated with a reduction of cryoglobulin level and complement activation in 5 cases and 6 cases, respectively. This study suggests that SPN of pSS result from a cryoglobulin-mediated vasculitis.
8092939 [Morphology of the minor salivary glands in Sjogren's disease]. 1994 May Histochemical, immunohistochemical and histomorphometrical changes in sialadenitis have been studied on 33 biopsies of labial salivary glands (LSG) from patients with primary Sjogren's syndrome. The control group consisted of 10 biopsies from persons without oral cavity inflammatory diseases. A direct relation was established between xerostomia stage and the degree of LSG parenchyma substitution for the inflammatory cell infiltrate. Dependence of LSG tissue damage on local immunopathologic reaction was detected. With a highly active inflammatory reaction the changes were characterized by cellular hypersensitivity including inflammatory cell infiltrate (T-lymphocytes, macrophages, neutrophils), increased vascular permeability, destructive and proliferative changes of the intralobular ductuli and acini. Reduction in inflammatory activity was accompanied by a decrease in the number of macrophages and neutrophils, an increase in the number of plasma cells and mast cells, higher compensatory hypertrophy of the serous cells of semicircular mixed acini, secretion products status, as well as intralobular sclerosis and lipomatosis.
1379507 Development of Hodgkin's disease in the course of primary Sjögren's syndrome. 1992 Aug We report two patients with a definite diagnosis of primary Sjögren's syndrome who developed Hodgkin's disease. Clinical and laboratory features of this transformation comprised prolonged fever, the appearance of lymphadenopathy together with loss of serum autoantibodies and a reduction in serum gammaglobulin levels. We know of only one well documented case of such an association. From these observations, it seems reasonable to include Hodgkin's disease in the clinical spectrum of the lymphoproliferative disorders that may occur in the course of primary Sjögren's syndrome.
1633351 Sjögren's syndrome with hydronephrosis caused by pseudolymphoma. 1992 Apr A 67-year-old woman with Sjögren's syndrome was found to have left hydronephrosis and stenosis of the left ureter. Exploratory laparotomy disclosed a nodule at the ureteropelvic junction of the left ureter. Histopathological examinations of the biopsied specimen of this nodule showed lymphoid hyperplasia within the ureteral wall. Low dose prednisolone improved hydronephrosis and pseudolymphoma within several months. Hydronephrosis secondary to pseudo-lymphomatous infiltration of the ureter is a rare but, if properly treated, reversible complication of Sjögren's syndrome.
8295196 Breast cancer with systemic manifestations mimicking Still's disease. 1993 Oct We describe a case whose clinical features strongly suggested Still's disease, which led to the discovery of breast cancer. Our patient's symptoms consisting of fever, joint inflammation, pleuritis, and pericarditis, were initially resistant to high doses of steroids, and disappeared only after the cancer was removed, despite rapid tapering and cessation of steroid therapy. A paraneoplastic phenomenon seems probable.
9601239 Primary Sjogren's syndrome and rampant dental caries in a 5-year-old child. 1995 Sep A 5-year-old boy of Pakistani parents first presented with rampant dental caries and later developed bilateral parotid gland enlargement accompanied by intermittent nocturnal fever, anorexia, lethargy and weight loss. Investigations revealed anaemia, hypergammaglobulinaemia, elevated non-specific indices of inflammation, a reduced Schirmer's test value, lymphocytic infiltration and some atrophy of the parotid gland. The parotid gland enlargement, low Schirmer's test value and salivary gland biopsy showing non-focal lymphocytic infiltration led to the diagnosis of primary Sjogren's syndrome. The present case report highlights the need for health professionals to be aware that Sjogren's syndrome is a possible, if rare, predisposing cause of rampant dental caries in children.
7553062 [A case of hemophagocytic syndrome manifesting adult Still's disease and acute hepatitis]. 1995 Apr We report a 20 year-old woman with hemophagocytic syndrome. In February 1993, she developed high fever, arthralgia, salmon-like pink eruption, leukocytosis and splenomegaly. She was diagnosed as adult Still's disease and successfully treated with intravenous immunoglobulin and oral prednisolone. In September 1993, she was re-admitted to our hospital complaining of general fatigue and low grade fever and treated with oral prednisolone at a daily dose of 15 mg. On October 2, 1993, she suddenly developed high fever and salmon-like pink eruption on her leg followed by the marked increase of serum transaminase and LDH levels (GOT 3,270 IU/l, GPT 1,880 IU/l, LDH 5,480 IU/l) on October 7. Since hepatic failure progressed, we started methylprednisolone pulse therapy and plasmapheresis. However, because of the progression of pancytopenia caused by hemophagocytosis, the treatment with VP-16 was initiated. However, she died of DIC on November 2, 1993. Autopsy revealed submassive necrosis of the hepatocytes with moderate infiltration of histiocytes. She was retrospectively diagnosed as hemophagocytic syndrome whose manifestations are very similar to those in adult Still's disease and acute viral hepatitis.
8156293 The treatment of sicca features in Sjögren's syndrome: a clinical review. 1994 Mar Patients with SS often suffer considerable distress due to sicca symptoms and the complications of mucosal dryness. Although there are many topical treatments available, the literature on their use is scant. This paper describes the treatments available and suggests a rationale for the choice of product.
7962239 Superantigens and their role in disease. 1994 Nov 15 Because superantigens bind to extremely large numbers of T cells, they are useful for investigating the immune system. Moreover, they appear to have diverse pathologic roles. They may be responsible for massive cytokine release in toxic shock, activate self-reactive T cells in Kawasaki disease and rheumatoid arthritis, and participate in immune tolerance and deficiency.