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ID PMID Title PublicationDate abstract
15703951 Sjögren's syndrome in the community: can serology replace salivary gland biopsy? 2006 Feb BACKGROUND: It is relatively difficult in a community setting to perform salivary gland biopsy or reliable diagnostic tests for salivary gland involvement in a patient suspected to suffer from Sjögren's syndrome (SS). OBJECTIVE: To investigate whether anti-Ro/La antibodies are a good substitute for salivary gland biopsy in community patients suspected to suffer from SS. METHODS: Forty-one patients suspected as having SS due to dry eyes and mouth, articular complaints, and/or serological findings were examined for the presence of anti-Ro/La, and underwent minor salivary gland biopsy. RESULTS: Sixteen patients (39%) were classified as primary SS by the American-European Consensus Group criteria. Twelve subjects had anti-Ro/La antibodies and 11 subjects in this group had positive biopsy findings. Of 29 patients without anti-Ro/La antibodies, only four manifested positive biopsy findings. A significant association was found between the presence of anti-Ro/La antibodies and positive salivary gland findings characteristic for SS (p<0.0001, Fisher's exact test). CONCLUSION: These findings tend to support the suggestion that a patient suspected to suffer from SS in a community setting may be first tested for the presence of anti-Ro/La antibodies to confirm the diagnosis. Only those with a negative result for the presence of anti-Ro/La antibodies need to be referred for salivary gland biopsy.
18563612 Development and evaluation of transgenic rice seeds accumulating a type II-collagen tolero 2008 Dec Type II collagen (CII) in joint cartilage is known to be a major auto-antigen in human rheumatoid arthritis. Several animal model- and clinical-studies on tolerance-based immunotherapy for the arthritis have been conducted by administrating synthetic immunodominant peptides through an oral route. In the present study, to produce a tolerogenic peptide with therapeutic potential in transgenic rice plants, a gene construct producing glutelin fusion protein with tandem four repeats of a CII(250-270) peptide (residues 250-270) (GluA-4XCII(250-270)) containing a human T-cell epitope was introduced with a selection marker, hygromycin phosphotransferase gene (hygromycin-resistance gene) (hph), by co-transformation. Several transgenic plants with high and stable expression of gluA-4XCII ( 250-270 ), but no hph, were selected based on both DNA and protein analyses. The GluA-4XCII(250-270) fusion proteins were detected as both precursor and processed forms mainly in a glutelin fraction of rice endosperm protein extracts and in protein-body rich fractions prepared by density gradient ultracentrifugation. The amount of accumulated CII(250-270) peptide was immunochemically estimated to be about 1 microg per seed. Feeding DBA/1 mice the transgenic rice seeds (25 microg of the peptide per mouse a day) for 2 weeks showed tendencies lowering and delaying serum specific-IgG2a response against subsequent and repeated intraperitoneal-injection of type II collagen. Taken these together, the CII-immunodominant peptide could effectively be produced and accumulated as a glutelin-fusion protein in the transgenic rice seeds, which might be useful as pharmaceutical materials and functional food for prevention and therapy for anti-CII autoimmune diseases like human rheumatoid arthritis.
18700333 Effect of Pilocarpine on impaired salivary secretion in patients with Sjögren's syndrome. 2008 The aim of this study was to analyze resting whole saliva (RWS) and stimulated whole saliva (SWS) flow rates before and after administration of Pilocarpine in secondary Sjögren's Syndrome patients. Fifty-one patients (49 women, 2 men, mean age 61 years, range 38-85), all with a resting saliva < or =0.1 ml/min, participated. Volumes of RWS and SWS collected over periods of 15 and 5 min, respectively, using standardized protocols were measured and the same procedure was repeated after oral administration of Pilocarpine (0.7 mg per 10 kg body weight). The sample was then divided into two groups, according to those in whom Pilocarpine stimulation had caused RWS flow to reach >0.1 ml/min (responders) and those who remained at values < or =0.1 ml/min (non-responders). All participants completed a questionnaire related to general and oral health status, as well as their subjective intraoral complaints before and after administration of Pilocarpine. Thirteen patients (25%) were classified as non-responders and the remaining 38 (75%) as responders. No statistically significant differences between the non-responders and responders were detected as regards general health parameters or intake of medicines with anticholinergic affect. As regards intraoral subjective complaints, no difference between the groups was found before Pilocarpine administration. After administration of Pilocarpine, complaints were significantly fewer among the responders (p<0.01). Both groups exhibited a significant decrease of intraoral symptoms after administration of Pilocarpine (responders P<0.001 and non-responders P<0.05) compared to baseline. For the whole group, more severe intraoral complaints were significantly associated with a lower SWS (P<0.05), but not a RWS, rate at baseline. It is concluded that a subgroup of Sjögren patients do not respond to Pilocarpine stimulation. The clinical implications of this finding need further investigation.
18602295 Labial salivary gland biopsies in Sjögren's syndrome: still the gold standard? 2008 Sep OBJECTIVES: The accuracy and diagnostic benefits of the labial salivary gland (LSG) biopsy for Sjögren's syndrome (SS) have received mixed reviews. This study was conducted to assess (1) the inter-rater agreement among 5 pathologists, and (2) the relationship between biopsy findings and clinical disease parameters. STUDY DESIGN: Three oral pathologists (OP) and two surgical pathologists (SP) provided independent diagnoses, focus scores, and plasma cell characterizations for 37 LSG biopsies. Inter-rater reliability was assessed using percentage of overall agreement and intraclass correlation coefficients. Relationships between diagnoses and clinical parameters were assessed by nonparametric correlations. RESULTS: Overall agreement among the pathologists was poor, although the intra-specialty agreement was good. The ratings of OP were most highly correlated with serological measures, while those of SP were correlated with salivary flow rate and disease damage. CONCLUSION: Since the LSG biopsy can be the determining factor in SS diagnoses, these demonstrated inconsistencies merit further consideration.
18443740 Successful treatment with plasma exchange in adult-onset Still's disease with hyper-IL-18- 2008 Adult-onset Still's disease (AOSD) is a rheumatoid disorder characterized by high fever, polyarthritis, leukocytosis, hyperferritinaemia, and mild liver involvement. We describe the case of a patient with AOSD with severe liver dysfunction. His serum levels of interleukin-10 and 18 showed a similar trend to his disease activity. Drug lymphocyte stimulation tests were positive for three drugs in the patient. Hypercytokinaemia was controlled by plasma exchange therapy.
18078628 Severe systemic inflammatory response syndrome in a patient with adult onset Still's disea 2007 Sep Interleukin 1 (IL1) plays an important role in adult onset Still's disease. Anakinra (Kineret), a recombinant IL1 Receptor Antagonist (IL 1 RA) was therefore recently proposed in adult onset Still's disease with great efficacy. Anakinra appeared to be well tolerated and safe. The case of a patient with refractory adult onset Still's disease who experienced a Systemic Inflammatory Response Syndrome and Adult Respiratory Distress Syndrome requiring intensive care unit hospitalization 10 days after the introduction of anakinra is reported.
17786140 Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögr 2007 Jul BACKGROUND/AIMS: Patients with primary Sjögren's syndrome may present liver involvement. Our goals were to establish the prevalence of abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome and correlate their presence with other clinical and laboratory features. METHODS: Ninety-five patients with diagnosis of primary Sjögren's syndrome were studied. Data on gender, age, clinical features, liver biochemistries, tests of inflammation and autoimmunity, and concomitant diseases were collected. RESULTS: Forty-two patients (44%) had abnormal hepatic biochemistries, and of these 19 patients (20%) had clinical liver disease. Patients with abnormal hepatic biochemistries had higher frequency of autoimmune hypotiroidism, arthritis, vasculitis, Raynaud's phenomenon, higher sedimentation rate,and higher frequency of antinuclear and antimitochondrial antibodies than patients with normal liver biochemistries (P < 0.05 for each). Patients with clinical liver disease had higher frequency of arthritis, vasculitis, and higher frequency of antimitochondrial antibodies than patients without clinical liver disease (P < 0.05 for each). Twenty-one patients had diagnosis of a specific liver disease, such as hepatitis C virus infection (n = 11), autoimmune hepatitis (n = 2), primary biliary cirrhosis (n =5),nonalcoholic fatty liver disease (n = 2), and hepatitis B virus infection (n = 1). In half of patients with liver involvement a definitive cause could not be identified. CONCLUSION: Liver involvement is frequently found in patients with primary Sjögren's syndrome, and its presence is associated with clinical features of systemic disease, and markers of autoimmunity and inflammation. There may be a subgroup of patients with liver involvement secondary to primary Sjögren's syndrome.
17197530 Relation between corneal innervation with confocal microscopy and corneal sensitivity with 2007 Jan PURPOSE: An alteration in corneal innervation has been described in dry eye associated with diabetes mellitus, contact lens use, and LASIK. The purpose of this study was to evaluate whether dry eye not related to Sjögren's syndrome (NSDE) and dry eye related to primary Sjögren's syndrome (PSDE) are associated with an alteration of the corneal nerves and sensation. METHODS: Twenty-one patients with dry eye (10 NSDE and 11 PSDE) and 20 healthy volunteers were studied. Healthy volunteers were divided into two groups: one younger than 60 years (N<60) and the other 60 years of age or older (N> or =60). The study of the epithelium, stroma, and subbasal corneal nerves was performed with a confocal microscope. Mechanical, chemical, and thermal sensation was evaluated using the Belmonte noncontact esthesiometer. RESULTS: A statistically significant decrease in the number and density of subbasal nerves (P < 0.0001) and the density of superficial epithelial cells (P < 0.0001) was observed in dry eyes. The number and density of subbasal nerves was higher in the N<60 group. A significant decrease was found with respect to mechanical, chemical, and thermal sensitivity (P < 0.0001). Sensibility was better in the healthy eyes. A strong correlation was found between the density of superficial epithelial cells and the nerves and between the number and density of subbasal nerves and sensation (P < 0.001). CONCLUSIONS: The use of confocal microscopy and noncontact esthesiometry allow the detection of the presence of corneal neuropathy in patients with dry eye.
16876917 [Cystic lung disease associated with Sjögren's syndrome: 2 cases]. 2006 Aug INTRODUCTION: Cystic lung disease is characterised on chest iconography by foci of decreased lung density with definable and thinned walls (wall thickness<4 mm) and with length's diameter superior at 1 cm. Cystic lung disease is exceptionally associated with the Sjögren's syndrome; very few cases have been described. EXEGESIS: We report two cases of cystic lung disease associated with Sjögren's syndrome, one occurring in a Lupus-Sjögren's overlapping syndrome, and another revealing primary Sjögren's syndrome. CONCLUSION: The Sjögren's syndrome should be recognised as could be associated with Cystic lung disease; and latent Sjögren's syndrome should be researched in presence of cystic lung lesions.
19048255 Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. 2009 Sep Adult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement. Diagnosis is usually clinical and made after other diseases in the differential diagnosis are excluded. We herein report the case of a patient with a remarkable familial autoimmune background in whom adult Still disease started off with a diffuse intravascular coagulation, probably triggered by a macrophage activation syndrome, followed by an acute interstitial myocarditis, leading to a fatal complete atrioventricular block. This case highlights that AOSD represents a troubling condition and that it may suddenly get worse with life-threatening events.
18612931 Primary Sjogren's syndrome in men. 2008 Jul OBJECTIVE: To determine whether there were any clinical and biological differences between male and female patients with primary Sjogren's syndrome (pSS) in a large bicentric series of patient. METHODS: We studied 419 consecutive patients (mean age at onset 53.6 years, mean disease outcome 73 months) with pSS according to American-European criteria, attending two different Departments of Internal Medicine in France. The 42 (9%) male patients in this cohort comprised the male group described in this study. RESULTS: Extraglandular manifestations during the course of the disease were present in 37 (89%) of our male patients with pSS. The extraglandular manifestations were similar among the two groups except that the male patients showed a lower frequency of depression or asthaenia (5% vs. 20%, p = 0.014) compared with the females. A significantly greater percentage of women reported lymphopaenia (26% vs. 8%, p = 0.02) and leucopaenia (18% vs. 3%, p = 0.015) at onset, but thrombopaenia was more common in the male patients (21% vs. 6%, p = 0.001). Lymphoma development was slightly more common in the male patients, but with no statistical significance (10% vs. 3%, p = 0.06), and occurred earlier after the SS diagnosis (log rank test p = 0.04). CONCLUSION: Although pSS is typically a disease affecting women, clinicians should be aware that it may be diagnosed in male patients. Except for haematological presentation, we could not find any notable differences in clinical and immunological characteristics between male and female patients with pSS.
18199044 Adult-onset Still's disease presenting as fever of unknown origin in a patient with HIV in 2008 Feb 15 A 43-year-old African American man with known human immunodeficiency virus (HIV) infection was found to have adult-onset Still's disease manifesting as fever of unknown origin. In the era of highly active antiretroviral therapy, HIV-infected patients are preserving their immune status and, thus, must be evaluated in a manner similar to that for the general population.
19062576 [Overlap syndromes in rheumatology]. 2008 Overlap syndromes in rheumatology present a challenging problem reflecting clinical and immunogenetic heterogeneity of rheumatic disorders. Current statistics refers 25% of the connective tissue diseases to "overlap syndromes". There are three main groups of them, viz. (1) overlap forms of systemic connective tissue diseases, (2) mixed connective tissue disease (Sharp's syndrome), (3) undifferentiated rheumatic disease. Characteristics of these groups are presented based on the published studies and the original data on 100 patients with the most frequent forms of systemic connective tissue pathologies, such as systemic sclerodermias with polymyositis/dermatomyositis (68 patients) and rheumatoid arthritis (32 patients).
18431325 Osteoporosis: clinical features. 2008 Apr Clinical features associated with osteoporotic fractures include increased morbidity (pain, physical impairment, decreased quality of life), increased risk for new fractures (even within short-term) and increased mortality. Readily recognizable clinical features that indicate a high risk for fracture include age, gender, low body weight, history of fracture, familial history of fracture, severe immobilization, smoking, rheumatoid arthritis, use of glucocorticoids and clinical risks for falls. In addition, many patients with fractures and osteoporosis have pre-existing contributors to secondary osteoporosis, many of which are correctable.
18309488 A case of adult onset Still's disease with systemic inflammatory response syndrome complic 2008 Jul Prolonged spiking fever, an evanescent salmon-colored rash, arthralgia or arthritis, leukocytosis and organ dysfunction are characteristic of adult onset Still's disease (AOSD). A 25-year-old woman with fever lasting over 3 weeks presented to our clinic. The patient had a spiking fever, sore throat, tender lymph nodes, a fine pink-colored skin rash, arthralgia, myalgia with a high ESR, ferritin and elevated hepatic enzymes. NSAID and prednisolone were prescribed for AOSD with SIRS. After 4 days of therapy, with mild confusion, the patient went into status epilepticus lasting several hours and died after cardiovascular collapse. There has been only one case of status epilepticus associated with AOSD in the medical literature. Here we report a case of AOSD with SIRS complicated by fatal status epilepticus.
17660908 [The value of MRS in diagnosis of Sjoigren's syndrome in parotid gland]. 2007 Jun PURPOSE: The purpose of this study is to evaluate the value of MR sialography in diagnosis of Sjoigren's syndrome in parotid gland. METHODS: MRI and MRS were used to diagnose Sjoigren's symdrome and differentiate with other disease combined with clinical, serologic and histopathologic result. Meanwhile, the result of MRS was compared with sialograph. RESULTS: 25 cases were ultimately diagnosed as Sjoigren's syndrome among 32 cases by clinical, serologic, histopathologic and sialography or MRS method. 23 cases were presented typical image in MRS, and sensitivity was 92%; specificity 71.4% and accuracy 87.5%. Other different manifestations were found in 25 cases, such as: autoantibody 68%, focui lymphocytic infiltration 72%. 18 cases have the positive results between MRS and sialography and 15 cases (83.3%) have the same manifestations by degree classification. CONCLUSION: The Sjoigren's syndrome can be confirmed by clinical, serologic, histopathologic, and MRS or sialography. MRS is a reliable method to diagnosis and differentiated diagnose Sjoigren's syndrome with other disease.
20144051 Inhibitors targeting hepatocyte growth factor receptor and vascular endothelial growth fac 2006 May Amgen disclosed a series of 4-heteroaryloxy quinoline/quinazoline compounds as multiple kinase inhibitors, including hepatocyte growth factor (HGF) receptor tyrosine kinase c-Met and vascular endothelial growth factor (VEGF) receptor tyrosine kinase. These compounds are stated to have wide therapeutic applications for the treatment of a variety of cancers, hypertension, arteriosclerosis, myocardial infarction and rheumatoid arthritis.
17356998 Pericardial tamponade masquerading as septic shock. 2007 Feb A 53-year-old man with steroid dependent rheumatoid arthritis presented with fever and serous articular drainage. Oral antibiotics were initially prescribed. Subsequent hemodynamic instability was attributed to septic shock. Further evaluation revealed a pericardial effusion with tamponade. Pericardiocentesis of purulent fluid promptly corrected the hypotension. Proteus mirabilis was later isolated from both the infected joint and the pericardial fluid. This is the first report of combined Proteus mirabilis septic arthritis and purulent pericarditis. It documents the potential for atypical transmission of Gram-negative pathogens, to the pericardium, in patients with a high likelihood of preexisting pericardial disease. In immunocompromised patients, the typical signs and symptoms of pericarditis may be absent, and the clinical presentation of pericardial tamponade may be misinterpreted as one of septic shock. This case underscores the value of a careful physical examination and proper interpretation of ancillary studies. It further illustrates the importance of initial antibiotic selection and the need for definitive treatment of septic arthritis in immunocompromised patients.
17701919 NFKB and NFKBI polymorphisms in relation to susceptibility of tumour and other diseases. 2007 Dec Nuclear factor-kappaB (NF-kappaB) is responsible for the expression by regulating many genes for immune response, cell adhesion, differentiation, proliferation, angiogenesis and apoptosis. The function of NF-kappaB is inhibited by binding to NF-kappaB inhibitor (IkappaB), and imbalance of NF-kappaB and IkappaB has been associated with development of many diseases, including tumours. In this review, we focus on polymorphisms of the NFKB and NFKBI genes in relation to development of common inflammatory diseases including ulcerative colitis (UC), Crohn's disease (CD), rheumatoid arthritis, systemic lupus erythematosus, psoriatic arthritis, giant cell arthritis, type 1 diabetes, multiple sclerosis, celiac disease, and Parkinson's disease, as well as susceptibility of several cancers, such as oral squamous cell carcinoma, colorectal cancer (CRC), hepatocellular carcinoma, breast cancer and myeloma.
17603446 Total knee arthroplasty in patients with severe deformities due to rheumatoid arthritis. 2006 Apr 28 In RA patients with a high activity disease, pathological changes in the knee occur in 90% of cases in the end-stage period. These changes are often accompanied by severe deformities, such as flexion contracture, valgus deformity, and more rarely varus deformity. In order to provide soft tissue balance, these deformities require additional surgery before implantation of the prosthesis. In this article the authors describe different methods, depending on the nature of the deformities, such as posterior release and lateral soft-tissue release, which should be done in successive stages. The problem of the patella and loss of bone stock for reconstruction is briefly discussed.