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ID PMID Title PublicationDate abstract
10920687 [A case of Sjögren's syndrome and systemic sclerosis complicated with acute pancreatitis] 2000 Jun We report a case of Sjögren's syndrome (SS) and systemic sclerosis (SSc) complicated with acute pancreatitis. A 51-year-old woman had been diagnosed as SS in 1973. She noticed Raynaud's phenomenon in 1977. In 1988, interstitial pneumonia (IP) was pointed out and she was treated with methylprednisolone (mPSL) pulse therapy. Prednisolone (PSL) was gradually tapered to 3-5 mg daily and she visited our outpatient clinic in 1995. On her first admission to our hospital in 1996, she showed xerostomia, keratoconjunctivitis sicca, sclerotic skin changes of her distal extremities and face, thickening of her sublinguinal frenulum, and regurgitative esophagitis. She was positive with anti-SS-A and SS-B antibodies. She was diagnosed as SS and SSc. Radiographic and laboratory data also established the diagnosis of inactive IP, renal tubular acidosis (RTA) and chronic renal failure (CRF). In April 30th 1997, she was admitted to our hospital again with complaints of dyspnea, dysesthesia, epigastralgia and petechia. Active IP and mononeuritis multiplex were diagnosed, and petechia was considered to be associated with vasculopathy. Her serum amylase level was 891 mU/ml on admission and spontaneously increased to 2440 mU/ml on May 12th along with increase of fibrinogen degradation product, D-dimer and alpha 2 plasmin-plasmin inhibitor complex levels. Ultrasonography depicted swelling of her pancreatic head and the diagnosis of acute pancreatitis was made. She was treated with protease inhibitors and intravenous hyperalimentation for acute pancreatitis. mPSL pulse therapy (500 mg/day for 3 days) was instituted for IP and mononeuritis multiplex on May 22, followed by 50 mg of daily PSL. While IP and mononeuritis multiplex gradually improved by the high-dose steroid therapy, serum amylase level raised to more than 4293 mU/ml, suggesting the modification of pancreatitis by the treatment with steroid. Since she did not respond to the conservative therapy for acute pancreatitis, she was treated with plasmapheresis, which turned out to be very effective. However, she was suffered from fungal pneumonia and died of respiratory failure. As far as we know, only three cases of SS with acute pancreatitis have been reported so far. The immunopathological mechanisms of development of acute pancreatitis in our case, especially focusing on the significance of microvasculopathy and hypercoagulability, were discussed.
10390969 [Mucosa-associated lymphoid tissue lymphoma with Sjögren's syndrome]. 1999 Apr A 68-year-old woman presented with Sjögren's syndrome. Chest X-ray films disclosed consolidated shadows in the right S2 and an infiltration shadow in the right S8 with small nodules. Pathological examination of transbronchial lung biopsy (TBLB) specimens revealed lymphocytic infiltrations that stained positive with UCHL-1 and L 26 in immunohistochemical studies. Lung tissue specimens obtained by video-assisted thoracic surgery showed lympho-epithelial lesions with dense lymphocytic infiltration. Southern blot hybridization and polymerase chain reaction (PCR) assays demonstrated monoclonality and immunoglobulin heavy chain gene rearrangement. These findings yielded a diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma. The detection of rear-ranged genes encoding for immunoglobulin heavy chains is useful for the diagnosis of primary pulmonary lymphoproliferative disorders, especially malignant lymphomas.
9825932 Lymphoid complement of the human salivary glands: function and pathology. 1998 Aug In normal human salivary glands the Duct-Associated Lymphoid Tissue (DALT) is poorly developed. In contrast, in the course of autoimmune disorders, typified by Sjögren's syndrome (SS), organized lymphoid accumulations are formed around the ducts. B cell-dependent zones with secondary follicles and T cell-dependent zones with HEV are detected in these lymphoid structures. In addition, the duct epithelium is infiltrated by abundant lymphocytes. A persistent antigenic stimulation may lead to development of B-cell Mucosa-Associated Lymphoid Tissue (MALT) lymphomas that, in low-grade cases, maintain the lobular organization of normal and of SS salivary glands.
11385268 Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjögren's sy 2001 Sjögren's syndrome (SS) is an important but poorly recognized cause of peripheral neuropathy. Several forms of peripheral nerve dysfunction occur, including trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensorimotor polyneuropathy and pure sensory neuronopathy. The pathological findings vary and the definite treatment is not known. Here we present 4 cases of acute ataxic sensory polyneuropathy with SS, and the experience of treatment with plasmapheresis (PP). The 4 patients were all females; ages ranged from 30 to 58 years. All had prominent loss of kinesthetic and proprioceptive sensation. The course ranged from acute to subacute onset. Patients were treated with 5-9 sessions of PP. Two patients with initiation of treatment within 2 weeks of onset showed dramatic and sustained responses after PP, while the other 2 had no detectable effects. Our experience showed that PP should be considered in patients who present with sensory neuropathy associated with SS, and the treatment should be given as early as possible.
10930601 Sjögren's syndrome with acute transverse myelopathy as the initial manifestation. 2000 Jun 15 This is the first report of a patient with acute transverse myelopathy as the initial manifestation of primary Sjögren's syndrome (SS). The patient developed tetraparesis and sensory disturbance within 6 days of onset. Spinal MRI showed an extensive intraparenchymal lesion with high T2-weighted signal intensity, gadolinium enhancement, and cord swelling. Although the patient did not show clinical sicca symptoms, primary SS was diagnosed on the basis of clinical tests on lacrimal and salivary glands which showed high levels of autoantibodies. Treatment with prednisone improved motor and sensory symptoms and resulted in improvement of MRI findings. The present case suggests that acute transverse myelopathy can occur as an initial symptom of SS.
10549066 [Sjögren's syndrome and necrotizing sarcoid-like granulomatosis]. 1999 Sep We report the first description of a Sjögren's syndrome associated with nectrotizing sarcoid-like granulmatosis. A 62-year old woman was admitted for diagnostic exploration of fatigue, weight loss, and fever at 38 degrees C which had progressed for more than 3 months. Chest X-ray showed several pulmonary opacities. There was a history of tuberculosis and thyroiditis at the age of 20 years. Physical examination revealed a sicca syndrome. There was no objective evidence of respiratory disease. Her heart and chest were clear on auscultation. The patient had autoantibodies for SSa and SSb specific antigens. Minor salivary gland biopsy gave a score of 3 in the Chisholm classification. Chest X-ray and CT-scan showed diffuse infiltrative opacities which were dense and peripheral with no retraction. Bronchoalveolar lavage, and bronchial and transbronchial biopsies were non-specific. Surgical lung biopsy showed typical aspects of necrotizing sarcoid-like granulomatosis. After high-dose pulse corticosteroid therapy, the opacities disappeared with no recurrence under oral steroids at three years.
10229404 D-mannose and N-acetylglucosamine moieties and their respective binding sites in salivary 1999 Apr OBJECTIVE: Sjögren's syndrome (SS) is an autoimmune exocrinopathy. The mannose binding lectin (MBL), a pluripotent molecule of the innate immune system, is involved in the pathogenesis of autoimmune diseases. We investigated whether specific ligands for MBL and MBL related structures could be reliable markers in cases of SS. METHODS: The labial salivary glands of 19 patients fulfilling the diagnostic criteria for primary (n=11) and secondary SS (n=8) were studied. Seven healthy women served as controls. Computer assisted microscopy was employed to determine quantitatively the percentage of positive structures (acini, ducts, and interlobular connective tissue), the staining intensity, and the level of staining heterogeneity for 4 glycohistochemical probes including wheat germ agglutinin and concanavalin (Con A) as lectins, and mannose and N-acetylglucosamine as parts of neoglycoproteins. The data were evaluated by discriminant analysis. RESULTS: The data strongly suggest that MBL related structures, if not MBL itself, could play distinct roles in the pathogenesis of primary versus secondary SS. Further, quantitative determination of the level of expression of D-mannose and N-acetylglucosamine and their respective binding sites in labial salivary gland acini offers a powerful diagnostic tool for distinguishing primary from secondary SS. CONCLUSION: In SS labial salivary glands, determination of the level of acceptor sites for wheat germ agglutinin, Con A, D-mannose, and N-acetylglucosamine provides information on the roles played by glycoforms in SS. The methodology and data described in this paper should provide pathologists with objective diagnostic markers for SS. Our results should enhance the biological understanding of this pathology.
9469545 The ratio of albumin to lactoferrin in tear fluid as a diagnostic tool in primary Sjögren 1997 Oct PURPOSE: To compare the protein composition of tear fluid obtained from patients with primary Sjögren's syndrome, patients with other connective tissue diseases and control individuals. METHODS: SDS polyacrylamide gel electrophoresis followed by staining with Coomassie blue and Western blotting. RESULTS: Comparison revealed the presence of a characteristic difference in the ratio of intensity between the albumin and lactoferrin bands. An albumin:lactoferrin ratio above 2:1 was significantly more common in patients with primary Sjögren's syndrome than in patients with other connective tissue diseases or the controls. CONCLUSION: An albumin:lactoferrin ratio above 2:1 may therefore prove of use when diagnosing 1(0)SS. Used as a test, its sensitivity was 67% and its specificity 100%.
11642512 Soluble interleukin-2 receptor in Sjögren's syndrome: relation to main serum immunologica 2001 Our aim was to study sIL-2R relationship with main serum immunological and LSG immunohistochemical parameters, including surface antigen expression of immune activation, in 27 patients with primary SS. Serum sIL-2R levels were significantly higher in SS (p<0.00005), as well as in SLE (p<0.05) and RA (p< 0.000001) patients than in controls. In SS patients with abnormal slL-2R values (n = 7) we found higher levels of anti-SSB/La antibodies (p<0.05), IgM-RF (p<0.014) and CRP (p<0.003) with respect to those with normal sIL-2R values (n = 20). Moreover, sIL-2R levels correlated positively with those of anti-SSB/La antibodies (p<0.0037) and with CRP (p<0.008). The comparison of groups with (A) and without (B) abnormal slL-2R levels reveals a statistically different percentage of patients with foci number > 1 (86% vs 40%; p <0.047), and CD25 expression on lymphocytes (100% vs 40%; p < 0.008). The frequency (p < 0.025) of CD25 expression on lymphocytes was higher in group A than in group B. The frequency of CD25 expression on the infiltrates correlated not only with sIL-2R levels (p<0.047), but also with anti-SSB/La antibody values (p < 0.044), with Tarpley histological classes (p < 0.009) and with frequency of HLA-DR expression on lymphocytes (p<0.004) and on epithelial cells (p<0.002). The frequency of epithelial CD25 expression also correlated with that of epithelial HLA-DR (p<0.004). Our report suggests that slL-2R is linked to glandular involvement in primary SS.
11223737 Bilateral empyema caused by adult-onset Still's disease. 2001 Empyemas are usually of infectious origin. Noninfectious causes of empyema may be a considerable diagnostic challenge, as exemplified in the present case report. A 21-year-old male presented with high fever, sore throat and myalgias of 1 week duration. In the following days, bilateral pleural effusions developed, with cellular counts in the pleural fluid up to 117 x 10(9)/liter (98% neutrophils). Despite institution of empiric antibiotic therapy, spiking fever continued. All culture studies resulted in being negative. Following the report of a serum ferritin concentration of 6,975 microg/l, adult-onset Still's disease was diagnosed and successfully treated with anti-inflammatory drugs. This case adds adult-onset Still's disease to the list of noninfectious causes of empyema and underlies the value of measuring serum ferritin as a diagnostic tool.
10684035 [Histopathologic and immunochemical changes in Sjögren's syndrome]. 1997 Sep To research into the causes of sjögren's syndrome, we investigated the lacrimal gland tissues of 6 cases of sjogren's syndriome by using electronmicroscopy and immunochemical methods. The results revealed that at the early stage the lacrimal gland cells showed degeneration, irregular arrangement and many intracellular vacuoles. At the middle stage, some lymphocytes infliltrated into the gland tissues. And at the advanced stage, lymphocytes and collagenous fibers were there in substitution of gland cells.
9253975 Keratoconjunctivitis sicca and primary Sjögren's syndrome in a Danish population aged 30- 1997 Jun PURPOSE: To investigate the presence of keratoconjunctivitis sicca and primary Sjögren's syndrome a sample of 504 persons aged 30-60 years from Copenhagen was studied. METHODS: The presence of keratoconjunctivitis sicca and primary Sjögren's syndrome was estimated according to both the Copenhagen set of criteria and the preliminary European criteria. RESULTS: Symptoms of dry eye and oral dryness were very common and with no significant correlation to age or sex. Keratoconjuncitivis sicca was most frequent in persons aged 50-59 years and was equally common in men and women. Primary Sjögren's syndrome could only be diagnosed in one person according to the Copenhagen criteria (cut-off the Schirmer-1 test < or = 5 mm/5 min), and in three persons according to the preliminary European criteria. CONCLUSION: The frequency of keratoconjunctivitis sicca in persons aged 30-60 years in Copenhagen may be estimated to be 11% according to the Copenhagen criteria and 8% according to the preliminary European criteria. The frequency of primary Sjögren's syndrome in persons aged 30-60 years in Copenhagen may be estimated to be between 0.2% and 0.8% according to the Copenhagen criteria and between 0.6% and 2.1% according to the preliminary European criteria.
9213177 Aplastic anemia complicating Sjögren's syndrome. 1997 May A 47-year-old woman was referred to our hospital because of severe anemia and polyclonal gammopathy. She developed sicca syndrome after admission. Laboratory data revealed pancytopenia (white blood cells, 2,800/microliter; hemoglobin, 6.4 g/dl; platelets, 6.1 x 10(4)/microliter) and hyper gamma globulinemia (5.2 g/dl), and bone marrow was hypoplastic. Histology of the salivary gland showed infiltration of lymphocytes. We report a good response to immunosuppressive therapy in a rare case of aplastic anemia complicating Sjögren's syndrome.
10390602 Mizoribine and mycophenolate mofetil. 1999 Jul Both mizoribine (MZR) and mycophenolate mofetil (MMF) are immunosuppressive agents that inhibit the proliferation of lymphocytes selectively, via inhibition of IMPDH. MZR is a nucleoside of the imidazole class, isolated from the culture medium of the mold Eupenicillium brefeldianum M-2166. Although this compound has been found to have weak antimicrobial activity against Candida albicans, it has proved ineffective against experimental candidiasis. Unlike azathioprine, this compound is not taken up by nucleic acids in the cell. Instead, after phosphorylation MZR-5 -monophosphate inhibits GMP synthesis by the antagonistic blocking of IMPDH (Ki = 10(-8)M) and GMP- synthetase (Ki =10(-5) M). The drug has been found to inhibit both humoral and cellular immunity, and on this basis it was developed in Japan as an immunosuppressant. MZR has been shown in animal experiments to lack oncogenicity, and has been shown clinically to be associated with a low incidence of severe adverse reactions. MZR has been registered in Japan for the prevention of rejection in renal transplantation, and for the treatment of lupus nephritis, rheumatoid arthritis and the nephrotic syndrome. MMF is the morpholinoethyl ester prodrug of mycophenolic acid (MPA), which was first isolated in 1896 from the culture media of several Penicillium species. MPA has been evaluated for its unique properties as an anticancer, antiviral, antifungal and antibacterial agent, as well as for its therapeutic use in psoriasis and rheumatoid arthritis. MMF was designed to enhance the oral bioavailability of the parent compound. After beneficial effects were observed in animals, the clinical efficacy of MMF as an immunosuppressant in renal transplantation was studied in the United States. In 1995 the US Food and Drug Administration (FDA) approved the use of MMF for the prevention of rejection in renal transplantation, the drug also available on a number of European markets.
9536822 Hepatitis G virus infection in primary Sjögren's syndrome: analysis in a series of 100 pa 1998 Jan OBJECTIVE: To determine the prevalence and clinical significance of hepatitis G virus (HGV) infection in a large cohort of patients with primary Sjögren's syndrome (SS). PATIENTS AND METHODS: The study included 100 consecutive patients (92 female and eight male), with a mean age of 62 years (range 31-80) that were prospectively visited in our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Two hundred volunteer blood donors were also studied. The presence of HGV-RNA was investigated in the serum of all patients and donors. Additionally, HBsAg and antibodies to hepatitis C virus were determined. RESULTS: Four patients (4%) and six volunteer blood donors (3%) presented HGV-RNA sequences in serum. HGV infection was associated with biochemical signs of liver involvement in two (50%) patients. When compared with primary SS patients without HGV infection, no significant differences were found in terms of clinical or immunological features. HCV coinfection occurs in one (25%) of the four patients with HGV infection. CONCLUSION: The prevalence of HGV infection in patients with primary SS is low in the geographical area of the study and HCV coinfection is very uncommon. HGV infection alone does not seen to be an important cause of chronic liver injury in the patients with primary SS in this area.
10586590 [Primary Sjögren's syndrome with lymphocytic interstitial pneumonia and pulmonary multipl 1999 Oct We report a case of primary Sjögren's syndrome with lymphocytic interstitial pneumonia and multiple cystic lesions. The patient was a 64-year-old woman. Abnormal chest shadows were detected by x-ray and computed tomographic (CT) examinations. The patient had no family history of disease and had never smoked. She had complained of dryness in the eyes and mouth for about 10 years. Laboratory tests were positive for anti-nuclear antigen, anti-SS-A antigen, and anti-SS-B antigen. Sialography revealed marked destruction of the salivary glands, yielding a diagnosis of Sjögren's syndrome. Chest X-ray films and CT scans showed multiple cystic lesions in both lungs, measuring from a few mm to 3 cm in diameter, as well as fine centrilobular nodules. Slight anemia and hyper gamma globlinemia were also detected. Pulmonary function tests showed mild obstructive disturbance. Bronchoalveolar lavage analysis disclosed an elevated lymphocytic fraction (28.6%), but transbronchial lung biopsy provided no adequate specimens for diagnosis. Thoracoscopic lung biopsy specimens demonstrated marked infiltration of lymphocytes and histiocytes through the interstitium of alveolar walls and peri-bronchovascular sheath, with some lymphoid follicles. The overall appearance was compatible with lymphocytic interstitial pneumonia. The cysts themselves were nonspecific, and no cellular infiltration was noted in the cyst walls. Because of the predominantly peribronchial distribution of the lesions, we suspected that the cysts were formed by the check valve mechanism. However, no definitive evidence was obtained.
10028847 [Multifocal nodular AL amyloidosis in primary Sjögren's syndrome]. 1998 Dec Primary Sjögren's syndrome (primary SS) is characterized by lymphocytic and plasma cell infiltration of the lacrimal and salivary glands, sometimes extending to extraglandular sites. An increased incidence of B-cell lymphoproliferative disorders has been observed in patients with primary SS. We recently studied an unusual case of primary SS associated with multifocal nodular AL amyloidosis (amyloidomas), located in the lower respiratory tract and oral mucosa. A 66-year-old woman with primary Sjögren's syndrome since 1966 was hospitalized in 1996 because of multifocal nodular shadows on a chest radiograph. An open lung biopsy specimen was obtained, revealing a large mass of amorphous eosinophilic material with green birefringence when stained with Congo red. Numerous plasma cells surrounded the amyloid deposits, aggregated in nodules and around blood vessels. The cytoplasm of these cells stained monotypically with anti-lambda light chain, using a peroxidase technique. Amyloid material did not stain with antibodies directed against kappa and lambda light chains, or against IgG, IgA and IgM heavy chains. Immunofixation of the serum and concentrated urine did not demonstrate monoclonal immunoglobulins. AL-amyloidomas might be a manifestation of local immunoglobulin production and amyloid formation within extramedullary plasmacytoma. This plasmacytoma might be burned out, namely, overcome by the deposits and no longer recognizable.
9498030 Subacute cutaneous lupus erythematosus: two cases of delayed diagnosis. 1998 Jan Two cases of subacute cutaneous lupus erythematosus are described. Features which delayed diagnosis are discussed. The potential clinical and histopathological overlap between this condition and erythema multiforme is emphasized, drawing attention to the histological features which allow distinction. The possibility that some cases of Rowell's syndrome may be manifestations of subacute lupus erythematosus is discussed.
10219125 Maintenance of prosthetic treatment in a geriatric patient--case report. 1998 A 76-year old female patient came to the Specialized Clinic for Long-Lasting Dentures of the Dental School of Ribeirão Preto, USP, for prosthetic treatment for the correction of the absence of several teeth. The patient's medical history and examination revealed the presence of psoriasis that had progressed to a rheumatoid arthritis-like condition causing motor deficits such as difficulty in picking up and holding a toothbrush. We proposed two acrylic removable partial dentures, with a chromium-cobalt metal support planned for the lower denture. For the maintenance of oral hygiene we proposed an acrylic resin device coupled to the toothbrush in order to increase the volume of the toothbrush, and consequently facilitate tooth brushing. This device, although simple, has permitted the patient to have a more independent and healthy life style.
11065256 Type II diabetes mellitus and primary Sjögren's syndrome complicated by pleural effusion. 2000 Nov A 73-year-old man was admitted to our hospital because of pleural effusion and nephrotic syndrome. Sjogren's syndrome (Sjs) was diagnosed based on a positive test for antibodies to Ro and La, and the result of labial salivary gland biopsy. The pleural effusion showed a high number of lymphocytes and high titers of antibodies to Ro and La. By immunohistochemistry, it was determined that infiltrating CD3+ cells predominated over infiltrating CD20+ cells in the pleura. Nephrotic syndrome was also present, which, as confirmed by renal biopsy was due to advanced diabetic nephropathy. Here, we report a case of Type II diabetes mellitus and primary Sjs complicated by pleural effusion, discuss the available treatment for pleural effusion.