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ID PMID Title PublicationDate abstract
20711100 Adult-Onset Still's Disease and cytomegalovirus infection. 2010 Apr We present a case of a previously asymptomatic 34-year-old man that presented to the emergency department with two weeks of fever, arthralgia of the wrists and knees and sore throat. He was diagnosed with cytomegalovirus (CMV) mononucleosis. The patient remained symptomatic in the 5 following months. After an extensive workup to exclude other clinical conditions, a liver biopsy was performed and CMV hepatitis was diagnosed. He started valganciclovir therapy. Approximately one year after the initial complaints, the patient remained ill and presented clinical criteria compatible with Adult Onset Still's Disease. The patient had a marked improvement after institution of prednisolone, an effect that has been sustained during the following months.
20391511 Accurate detection of changes in disease activity in primary Sjögren's syndrome by the Eu 2010 Apr OBJECTIVE: To assess and compare the sensitivity to change of the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI) with that of other primary Sjögren's syndrome (SS) disease activity indexes. METHODS: We abstracted 96 patient profiles, including data on 3 successive visits (visits 1-3), from the medical charts of patients with primary SS. Patient profiles were scored with the ESSDAI, SS Disease Activity Index (SSDAI), and Sjögren's Systemic Clinical Activity Index (SCAI). Thirty-nine experts assessed 5 profiles for whether disease activity had improved, worsened, or remained stable at visits 2 and 3. RESULTS: For improved patients, the standardized response means (SRMs) for all scores did not differ, and ranged from -1.08 to -1.38 between visits 1 and 2 and from -0.50 to -0.76 between visits 2 and 3. For patients with worsened activity, the SRMs between visits 1 and 2 and between visits 2 and 3 were +0.46 and +1.10 for the ESSDAI, -0.03 and +0.79 for the SSDAI, and +0.17 and +1.02 for the SCAI, respectively. For patients with stable activity, the SRMs between visits 1 and 2 and between visits 2 and 3 were 0.00 and -0.13 for the ESSDAI, -0.44 and -0.11 for the SSDAI, and -0.36 and +0.34 for the SCAI, respectively. CONCLUSION: For patients with improved activity, the 3 disease activity indexes showed similar, large sensitivity to change. However, the ESSDAI seemed to detect changes in activity more accurately than other disease activity indexes. Notably, for patients with stable activity, the ESSDAI did not show erroneous improvement.
19637801 [A rare case of primary Sjögren's syndrome complicated by secondary lymphoid interstitial 2009 Jul A 61-year-old woman was referred to our hospital because of an abnormal chest X-ray shadow. She was diagnosed as having primary Sjögren's syndrome based on sicca syndrome, polyclonal hyper-gamma-globulinemia, and positive results for anti-Ro/SS-A and anti-La/SS-B antibodies. Her chest CT scan revealed bilateral ground-glass shadows and septal thickning in the lower lung field (Fig. 2a). Biopsy specimens obtained by video-assisted thoracoscopy (VATS) showed a dense interstitial lymphoid infiltrate, including lymphocytes and plasma cells, predominantly in the alveolar septae (Fig. 3, a-c). Malignant lymphoma was excluded on the basis of immunohistopathological studies (Fig. 4). A diagnosis of lymphoid interstitial pneumonia was made according to the 2002 American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification (International Classification). After administration of prednisolone and azathioprin, the ground-glass shadows on the chest CT rapidly improved (Fig. 2b). The clinico-radiologic-pathologic diagnosis based on the VATS procedure and the International Classification is appropriate and effective for treating collagen vascular disease-associated interstitial lung disease.
19624971 [Pellucid marginal degeneration and corneal ulceration, associated with Sjögren syndrome] 2009 Jan CLINICAL CASES: two cases reported presented corneal topography with peripheral inferior corneal slimming (pachymetry), flattening of superior and central meridian, high astigmatism against the rule, corroborating clinical pellucid marginal degeneration prognosis. A rheumatologist and a dermatologist confirmed primary and secondary Sjögren's syndrome in each case. CONCLUSIONS: In the cases studied, the pellucid marginal degeneration was presented with inferior corneal slimming, progressive irregular astigmatism and decreased visual acuity. However, the patients were treated for a long time only for dry eye. The association between pellucid marginal degeneration, primary Sjögren syndrome and secondary Sjögren syndrome has not been described. In the analyzed cases, both pathologies were observed and caused a bad prognosis because the treatment required is very difficult.
20219783 Autonomic nervous dysfunction development in patients with primary Sjogren's syndrome: a f 2010 Jun OBJECTIVES: To investigate autonomic dysfunction (AD) development in patients with primary SS (pSS) and the associations between AD and clinical, inflammatory and serological features of pSS. METHODS: Twenty-seven patients with pSS, who had previously been evaluated for AD, were included in the study. The patients were studied at baseline and at follow-up by objective autonomic reflex tests (ARTs) and by the autonomic symptom profile (ASP) questionnaire, evaluating AD symptoms. The median follow-up time was 5 years for the ART and 4 years for the ASP variables. The results were compared with previously investigated healthy ART controls and population-based ASP controls. Fatigue, anxiety and depression were assessed by the profile of fatigue and by the Hospital Anxiety and Depression scale. RESULTS: Three of five ART variables as well as the ASP total score were significantly abnormal both at baseline and at follow-up in pSS patients in comparison with controls. When comparing ART and ASP results in pSS patients between baseline and follow-up, only the lowest diastolic blood pressure (lDBP) ratio significantly deteriorated during the follow-up period. The ART and ASP variables were not significantly correlated. However, the ASP total score significantly correlated with measurements of fatigue, anxiety and depression. CONCLUSIONS: Both objective signs and subjective symptoms of parasympathetic and sympathetic dysfunction were seen in pSS patients, both at baseline and at follow-up. During follow-up, only the lDBP ratio was found to significantly deteriorate. AD symptoms were significantly associated with fatigue, anxiety and depression.
19096778 Aquaporins in secretory glands and their role in Sjögren's syndrome. 2009 Salivary, lacrimal and pancreatic secretions are known to account for multiple physiological functions. These exocrine secretions are watery fluids containing electrolytes, and a mixture of proteins, and can be stimulated by a number of agonists. Since water movement is involved in exocrine secretion, aquaporins (AQPs) have been hypothesised to contribute to fluid production in exocrine glands. This chapter will focus on the expression, localisation and function of AQPs in salivary and lacrimal glands and pancreas. The role of multiple water and ion transporters and channels in exocrine fluid secretion will also be reviewed. Finally, this chapter will address the potential role of AQPs in Sjögren's syndrome.
20737186 Benefit and a possible risk of tocilizumab therapy for adult-onset Still's disease accompa 2011 Feb We report a 57-year-old female case of intractable adult-onset Still's disease (AOSD). Initial high-dose prednisolone therapy was ineffective, and macrophage-activation syndrome (MAS) manifested after one session of additional tocilizumab therapy. After successful treatment for MAS with lipo-dexamethasone and cyclosporin, tocilizumab therapy aided in the rapid reduction of the therapeutic steroid dose. Tocilizumab may be useful for maintenance therapy for AOSD, although its efficacy is unclear for the highly active phase of the disease.
20545046 Quantitative and visual evaluation of salivary and thyroid glands in patients with primary 2010 Jul PURPOSE: To evaluate quantitative and visual salivary gland scintigraphy in patients with primary Sjögren's syndrome (SS) and to compare the scintigraphic findings with clinicopathological features of the salivary, lacrimal and thyroid glands. METHODS: Twenty-seven patients with primary SS and 10 healthy age-matched and sex-matched individuals as a control group were included in this study. The diagnosis of SS was made on the basis of the classification criteria of the American-European Consensus Group. The patients with primary SS were divided into two groups as early-stage and advanced-stage according the labial biposy scores (16 early-stage and 11 advanced-stage). After the administration of 370 MBq Tc-99m pertechnetate, static images were obtained at 20 min and also after lemon stimulation. Functional parameters for the parotid, submandibular, and thyroid glands were calculated. Schirmer-I, tear film break-up time and Rose Bengal tests were evaluated. RESULT: Statistically significant differences existed for the functional parameters of the parotid and submandibular glands between the early-stage and advanced-stage groups. The submandibular glands were more frequently affected than the parotid glands. Asymmetric uptake pattern was more common in the parotid glands during the early-stages of primary SS. When compared with the control group, the thyroid uptake ratio of the early-stage primary SS and advanced-stage group was not significantly different. According to the results of the ophthalmological tests, there was a statistically significant difference between patients with advanced-stage and early-stage disease. There were no significant correlations between functional parameters of salivary gland and any of the ophthalmological tests. CONCLUSION: The results indicate that quantitative salivary gland scintigraphy may be a useful method for evaluating salivary gland dysfunction and also for determining disease severity in primary SS. Asymmetric activity pattern in the parotid glands commonly observed during the early stages of the disease may be a predictor of progression. In addition, the histopathological stage is related not only to the scintigraphic findings of the salivary glands but also to lacrimal gland function in primary SS. However, thyroid uptake seemed to be unaffected in patients with both early-stage and advanced-stage disease.
19158115 Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with 2010 Jan OBJECTIVES: To assess subclinical central nervous system (CNS) involvement in primary Sjögren syndrome (pSS), by comparing standard brain MRI, in-depth neuropsychological testing and (99m)Tc-ECD brain single-photon emission computed tomography (SPECT) of patients with pSS with matched controls. METHODS: 10 women (<55 years old), with pSS defined using European-American criteria, presence of anti-SSA and/or anti-SSB antibodies and no history of neurological involvement were prospectively investigated, and compared with 10 age- and sex-matched controls. All subjects underwent, within 1 month, brain MRI, neuropsychological testing, including overall evaluation and focal cognitive function assessment, and (99m)Tc-ECD brain SPECT. RESULTS: (99m)Tc-ECD brain SPECT abnormalities were significantly more common in patients with pSS (10/10) than controls (2/10; p<0.05). Cognitive dysfunctions, mainly expressed as executive and visuospatial disorders, were also significantly more common in patients with pSS (8/10) than controls (0/10; p<0.01). Notably, between-group comparisons enabled a significant correlation to be established between neuropsychological assessment and (99m)Tc-ECD brain SPECT abnormalities in patients with pSS (r(s) = 0.49, p<0.01). MRI abnormalities in patients and controls did not differ significantly. CONCLUSIONS: Neuropsychological testing and (99m)Tc-ECD brain SPECT seem to be the most sensitive tools to detect subclinical CNS dysfunction in pSS. The strong correlation between cortical hypoperfusion in (99m)Tc-ECD brain SPECT and cognitive dysfunction suggests an organic aetiology of CNS dysfunction in pSS. These data should be confirmed in a larger study.
19300358 Presence of fibronectin peptides in saliva of patients with Sjögren's syndrome: a potenti 2009 Aug 1 OBJECTIVE: The purpose of this preliminary study was to monitor the degree of destruction of salivary glands in Sjögren disease by the detection of fibronectin peptides in patients' saliva. STUDY DESIGN: The sample consisted of 10 subjects divided in 2 groups, one with Sjögren disease and a control group. Saliva samples were submitted to an inmunodetection analysis. In addition, non pathological salivary glands, obtained from 2 subjects who underwent minor oral surgery, were incubated with leukocyte homogenates and analysed to compare the obtained fragments. RESULTS: The inmunodetection analysis of Sjögren saliva revealed multiple protein bands, including fibronectin, that were not present in saliva from healthy individuals. The inmunostained bands varied depending on the course of the disease, showing more fibronectin fragments in an active phase. Furthermore, results obtained from the non-pathological glands incubated with leukocyte homogenates were similar to those revealed in saliva from Sjögren patients. CONCLUSION: The presence of fibronectin peptides in Sjögren patients' saliva can constitute a method to monitor activity in Sjögren's disease.
19214349 Unraveling the pathophysiology of Sjogren syndrome-associated dry eye disease. 2009 Jan Sjogren syndrome (SS) is one of the most common autoimmune diseases. Early clinical manifestations of SS are primarily decreased tear and saliva secretion, leading to dry eye and dry mouth syndromes, but in its later stages, it can become systemic, even resulting in B cell lymphomas. The use of new animal models, coupled with new technologies, is providing exciting insights into the pathogenesis, genetic predisposition, and, possibly, early diagnosis of SS. This article reviews newly described features of SS identified in experimental animal models and their relationship to human disease. New technologies, such as genomics and proteomics, may permit identification of potential candidate genes and biomarkers for disease diagnosis. Current studies using appropriate animal models in parallel with studies of human subjects are rapidly establishing a foundation for new intervention strategies that go beyond merely treating symptoms.
20037747 Marked effect and steroid-sparing ability of anakinra on a patient with refractory adult-o 2010 Apr We report a case of refractory adult-onset Still's disease (AOSD) successfully controlled with anakinra, an interleukin-1 (IL-1) receptor antagonist. The patient was a 23-year-old Japanese woman with AOSD who could not be induced into remission despite of two courses of pulsed methylprednisolone followed by high-dose glucocorticoid administration in conjunction with high-dose intravenously administered gamma-globulin and methotrexate. To the best of our knowledge, this is the first case report in Japan of AOSD remission induced with anakinra.
19474780 [Primary Sjögren syndrome and inclusion body myositis]. 2009 Apr The authors report the case of a 69-years-old woman with Sjögren's syndrome. After 8 years of disease she developed muscle weakness and the diagnosis of inclusion body myositis was established. This is a rare association. The patient had a good response to the immunosupressive treatment. Similar cases can be found in the literature and there seems to be a subset of inclusion body myositis associated with autoimmune diseases that shows a better response to treatment and a more favourable prognosis.
19185937 A rare case of reversible constrictive pericarditis with severe pericardial thickening in 2010 Oct 8 A 21-year-old male German patient, was referred to our intensive care unit from a peripheral hospital with severe septic illness and fever. The echocardiographic investigation revealed a massive pericardial thickening of at least 20 mm mainly located posterolateral and inferior, without typical pericardial effusion. An inspiratory diastolic septal shift to the left, as well as pathological respiratory PW-Doppler profiles of mitral and tricuspid inflow velocities could clearly been detected in the examination. The patient describes at the interview in our intensive care unit, that he has previously been well, without any symptoms of cardiac failure or joint pain. We diagnosed an adult onset Still's disease (AOSD). Thus, prednisone pulse-therapy was initiated with 250 mg and reduced stepwise in the following days. All the symptoms improved dramatically within a few days. The documented pericardial thickening showed a significant reduction to approximately 6 mm already after 4 days of therapy AOSD should not be forgotten in the differential diagnosis of pericardial disease due to its potentially life threatening complications. Usually, echocardiography reveals typical pericardial effusion. In this case the patient presented with an impressing, atypical thickening of the pericardium in the posterolateral region, hardly ever seen in AOSD.
18955278 Tibetan Medicated-Bath Therapy may Improve Adjuvant Arthritis in Rat. 2009 Jun Tibetan medicated-bath therapy has been applied to patients with rheumatoid arthritis for centuries. However, the detailed action mechanism of Tibetan medicated-bath therapy on the morphology and function of joints remains unknown. We designed our investigation to evaluate the efficacy of Tibetan medicated-bath therapy on adjuvant arthritis (AA) of rats in comparison with water-bath and dexamethasone administration. AA was induced by intradermal injection of Mycobacterium butyricum suspended in sterile mineral oil. The control animals were similarly injected with sterile vehicle. Eight days after injection, rats were treated with fresh-water bath, Tibetan medicated-bath (40 degrees C, 15 min) or intramuscular injection with dexamethasone for 21 consecutive days after which we evaluated the severity of arthritis visually and microscopically and measured serum interleukin (IL)-6 and tumor necrosis factor (TNF)-alpha levels. While arthritis did not significantly change after water-bath treatment, the Tibetan medicated-bath and dexamethasone groups showed diminished joint swelling and alleviation of, inflammatory cell infiltration and the destruction of bone and cartilage. Serum IL-6 and TNF-alpha levels significantly decreased. Our results demonstrated that Tibetan medicated-bath therapy exerted a reliable effect on rat adjuvant arthritis, which may be involved in the inflammatory cytokines, IL-6 and TNF-alpha. Our data provide evidence for clinical use of Tibetan-medicated bath therapy for arthritis patients.
20084945 [Anti-inflammatory effect of Qufeng Zhitong capsule on the collagen-induced arthritis rat] 2009 Nov OBJECTIVE: To investigate the role of anti-inflammatory effect and it's mechansim of Qufeng Zhitong capsule on the collagen-induced arthritis rat through the study of arthritis, joint inflammation and joint erosions. METHODS: Fifty male SD rat were assigned to 5 groups: normal control group, arthritis model group, and Qufeng Zhitong capsule treatment group (including low, medium and high doses), each group contains 10 rats. Arthritis were induced by immunization with type II collagen (CII) in complete Freund's adjuvant (CFA). After administered Qufeng Zhitong capsule 2 weeks, the volume of back foot of arthritis rat were detected, and the histology changes of the ankle joints of the arthritis rat were studied. The serum levels of IL-10 and TNF-alpha were measured by ELISA. RESULTS: After treated with Qufeng Zhitong capsule, the swelling of the paw of the rats were significantly decreased compared with the controls. Histology study demonstrated that the Qufeng Zhitong capsule treatment could reduce the synovium hyperplasia and inflammatory cell infiltration, the inhibitory effects were in accordance with the doses of the drug. The serum levels of pro-inflammatory cytokine of TNF-alpha in the arthritis rat were not changed significantly, while the serum levels of anti-inflammatory cytokine of IL-10 were elevated significantly after treated with Qufeng Zhitong capsule. CONCLUSION: Qufeng Zhitong capsule can inhibit the synovium hyperplasia and cartilage erosion by stimulating the serum levels of anti-inflammatory cytokine of IL-10 and suggest it can be a candidate of treatment of rheumatoid arthritis.
19758231 Treatment of rat adjuvant arthritis with flavonoid (Detralex), methotrexate, and their com 2009 Sep In both adjuvant arthritis and rheumatoid arthritis, edema and inflammation appear in synovial joints. Edema or effusion reflects an imbalance in lymph dynamics. Purified micronized flavonoid fraction is mainly used in the treatment of chronic venous insufficiency. This compound improves lymphatic drainage with a significant increase in lymphatic flow and lymphatic pulsality. It is suggested that the beneficial effect of purified micronized flavonoid fraction may be involved in the treatment of adjuvant arthritis in rats. In this study treatment of adjuvant arthritis in rats with Detralex, methotrexate, and their combination were evaluated. Groups of rats with adjuvant arthritis were treated with methotrexate (0.6 mg/kg/week), Detralex (20 mg/kg/day), and their combination for 50 days from adjuvant application. Hind paw swelling, arthrogram scores, serum albumin level, serum nitrite/nitrate concentrations, and whole-body mineral density were evaluated as markers of inflammation and destructive changes associated with arthritis. Long-term prophylactic treatment with low-dose methotrexate significantly inhibited the markers of both inflammation and arthritis. Detralex administered alone slightly decreased both the hind paw swelling and the arthritic score. Other inflammatory and arthritic markers were not significantly influenced. However, Detralex combined with methotrexate markedly potentiated the beneficial effects of methotrexate, which resulted in a more significant reduction in hind paw swelling, arthritic scores, and serum concentrations of nitrite/nitrate. Interestingly, the arthritis-induced decrease of bone mineral density in AA rats was significantly lower only in the group treated with the combination of Detralex and methotrexate. Our results indicate that Detralex increased the therapeutic efficacy of methotrexate basal treatment in AA. We suggest that this may be related to the beneficial effect of Detralex on microcirculation, especially on venules and lymphatic vessels.
25903029 Diagnosis and Treatment of Type II Necrotizing Fasciitis in a Child Pres 2009 Mar  Pyoderma gangrenosum (PG) is associated with systemic disease, mostly rheumatoid arthritis (RA) and inflammatory bowel disease (IBD), in many patients (more than 50%). Lesions associated with arthritis are often ulcerative. Although these lesions typically affect the lower limbs, they can also affect the entire body. Successful therapy involving monoclonal antibodies seems to favor an autoimmune etiopathogenesis that has disorders of neutrophils' chemotactic activity and interleukins, which are acted upon by TNF-α cytokines. The ulcers grow rapidly, exacerbate after trauma, and necrosis can invade each skin layer up to the fascia. Therefore, debridement is contraindicated because it introduces the so-called "pathergy" mechanism. The diagnosis is quite difficult and is often made late due to the lack of indicative clinical and laboratory findings. The following is a report of a case of multiple, ulcerative, PG ulcers induced by arthritis. The ulcers occurred over several occasions with large and aggressive necrosis reaching the osseous plane in the heel and elbow. The high IgE values (between 2000 UI/mL and 3000 UI/mL) suggested that a type I immunitary reaction, such as in-skin anaphylaxis, was involved. Nevertheless, the antigen remains unknown and the genesis may be multifactorial. A corticosteroid (prednisolone) was the first-line systemic treatment used in this case and caused rapid improvement. Further investigations will be necessary to understand the meaning of this immunologic disorder .
26238621 Proteomics: New insights into rheumatic diseases. 2009 Feb Tremendous advances undergone in electrophoresis, chromatography, and MS have led proteomic research to unprecedented achievement over the last decade. Proteomics is presently employed for assessing protein expression levels, for monitoring cellular activities and for determination of biochemical pathways, revolutionizing the way we study disease by opening up the possibility to decipher the pathogenesis of clinical manifestations. Over 200 disorders including osteoarthritis (OA), rheumatoid arthritis (RA), and osteoporosis are considered rheumatic diseases (RDs), which affect the musculoskeletal system (joints and other supporting structures of the body such as muscles, tendons, ligaments, and bones) and are a leading cause of disability among older adults. Despite that an autoimmune origin has been proposed for some RDs like RA, the pathogenesis of most of these diseases is still unclear. Therefore, proteomic research on RDs, notably OA and RA, can help clarify underlying disease mechanisms, develop biomarkers to improve early detection, measure response to treatment, and devise new therapies. Achievements in the field of proteomics research on RDs are summarized in this work.
20582714 Amebic monocyte locomotion inhibitory factor peptide ameliorates inflammation in CIA mouse 2010 Dec OBJECTIVE AND DESIGN: Monocyte locomotion inhibitory factor (MLIF), an amebic peptide with antiinflammatory properties, was evaluated in collagen-induced arthritis (CIA) to test its effects on the onset and acute inflammatory response of arthritis. MATERIAL: DBA1/J mice at 8-10 weeks of age were divided into four groups (eight mice per group). TREATMENT: The adjuvant group received Freund adjuvant, the CIA group was immunized with collagen II, the MLIF/CIA group received collagen II and MLIF, and the MLIF group received MLIF and Freund adjuvant. METHODS: All groups were evaluated clinically. Seven weeks after the collagen injection, at the peak of the clinical arthritis score, limb specimens were collected and histological studies and gene expression analysis using microarrays were performed. RESULTS: MLIF administered weekly as a preventive scheme delayed and reduced the severity of acute arthritis. MLIF induced gene changes in functional categories including adhesion molecules, matrix metalloproteinases, and inflammatory cytokines. CONCLUSIONS: MLIF could be an interesting new molecule to investigate in the field of rheumatoid arthritis pathogenesis research for its potential to prevent inflammation.