Search for: rheumatoid arthritis    methotrexate    autoimmune disease    biomarker    gene expression    GWAS    HLA genes    non-HLA genes   

ID PMID Title PublicationDate abstract
16365690 A multicenter study of patients with adult-onset Still's disease compared with systemic ju 2006 Sep Adult-onset Still's disease (AOSD) has often been regarded as the adult spectrum of systemic juvenile idiopathic arthritis (sJIA). The present study aims to compare the clinical and laboratory features, the disease course and the response to treatment in patients having AOSD with those having sJIA. Retrospective review of all available data that were filled out by adult and paediatric rheumatologists from six centers using a standard data extraction form was performed. A total of 95 patients with AOSD and 25 patients with sJIA were recruited for the study. The frequency of fever, rash, myalgia, weight loss and sore throat was higher in patients with AOSD. The pattern of joint involvement differed slightly. Laboratory findings were similar in both groups, except that liver dysfunction and neutrophilia were more common among adults. A multiphasic pattern dominated the childhood cases, whereas the most frequent course was a chronic one in adults. Corticosteroids and methotrexate were the most commonly employed therapy; however, chloroquine was another popular therapy in the adult group. We showed a difference in the rate of clinical and laboratory features between patients with AOSD and those with sJIA. AOSD and sJIA may still be the same disease, and children may simply be reacting differently as the result of the first encounter of the putative antigens with the immune system.
16357707 Adult-onset Still disease in southeast Brazil. 2005 Apr BACKGROUND: Adult-onset Still disease (AOSD) has been described all over the world. Clinical presentations and prognosis have varied in different studies. OBJECTIVE: The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil. METHODS: The clinical records of 16 patients were retrospectively studied to determine symptoms at diagnosis, follow up, and the medication prescribed. RESULTS: The mean age at onset was 30.8 years (range, 24-55 years; standard deviation [SD], 9.2 years) with a slight male prevalence (54.2%). All patients presented constitutional symptoms, fever, and skin rash. Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. All patients presented leukocytosis with a predominance of neutrophils. Elevated ferritin levels were observed in 56.3%, and these levels were normalized after disease remission. Initial treatments included nonsteroidal antiinflammatory drugs and low-dosage corticosteroids in all patients; 43.8% also needed methotrexate. In 25.0% of cases, a monocyclic disease was observed; others had recurrent episodes. After a follow up of 6.9 years (SD, 1.2 years), carpal ankylosis was the main articular sequel, observed in 53.6% of the patients. CONCLUSION: AOSD is rare in southeast Brazil. Although less severe systemic manifestations, like serositis and pneumonitis, were observed, reversible liver involvement was common; the frequency of recurrent disease and carpal ankylosis was higher than in previous studies.
16941204 Unusual sites of Salmonella osteoarthritis in patients with sickle cell disease: two cases 2007 Aug Salmonella osteoarticular infections involve mainly long bones such as the femur, tibia, and humerus in patients with sickle cell disease (SCD). We report here two unusual cases of Salmonella osteoarthritis affecting sacroiliac and sternoclavicular joints in two patients with SCD, one patient also being followed for rheumatoid arthritis. Because of misleading presentation, diagnosis of septic osteoarthritis in patients with SCD requires a high index of suspicion and an early treatment.
16900301 Purtscher's-like retinopathy as an initial presentation of adult-onset Still's disease: a 2007 Jul Adult-onset Still's disease is a multisystem inflammatory disorder of unknown etiology and is characterized by high, spiking fever, arthritis, evanescent maculopapular rash, myalgia, serositis, leukocytosis, and involvement of various organs including the eyes. The ocular manifestations have been described including orbital pseudotumor, ptosis, and diplopia with orbital pain but never Purtscher's-like retinopathy. We describe a 21-year-old male patient with adult-onset Still's disease who developed the Purtscher's-like retinopathy. To our knowledge, this is the first reported adult-onset Still's disease patient with Purtscher's-like retinopathy as the initial presentation.
17110311 Immunohistopathology of Sjögren's syndrome. 2006 Nov Sjögren's syndrome (SS) is characterized by keratoconjunctivitis sicca and xerostomia, which occur in an autoimmune lacrimal and salivary gland disease characterized by lymphocyte infiltrates of exocrine glands and/or Sjögren's syndrome autoantibody production. It has been reported that aquaporin-5 distribution is abnormal in SS, perhaps as a result of paracrine effect of TNF-alpha. Also the neurogenic regulation of the salivary gland is impaired in SS. Apart from functional changes, the syndrome is also characterized by structural abnormalities of the secretory acinar apparatus. The acinar basement membrane is abnormal as it lacks laminin alpha1 chain, which may impair its capability to induce the progenitor cells to differentiate to acinar cells. CRISP-3 and TMPRSS-2 can be used as androgen markers and LIV-1 and Cyr61 as estrogen markers to study the sexual dimorphism of the salivary glands. Patients with SS seem to have low concentrations of dehydroepiandrosterone, which may predispose women and the exocrine glands to this syndrome.
16257401 Lysosomal enzyme activities: new potential markers for Sjögren's syndrome. 2005 Dec OBJECTIVE: To evaluate the changes in lysosomal enzyme activities in leukocytes of patients with Sjögren's syndrome. METHODS: Leukocytes were obtained from 38 patients with Sjögren's syndrome and 36 healthy subjects. The activities of the following glycosidases were measured: alpha-glucosidase (AGU), beta-galactosidase (BGA), alpha-mannosidase (AMAN), beta-glucuronidase (GCU), beta-hexosaminidase (HEX), and the following proteases: cathepsin B (CATH B), dipeptidyl peptidase I (DPP I), cathepsin H (CATH H), dipeptidyl peptidase II (DPP II), tripeptidyl peptidase I (TPP I), and cathepsin D (CATH D) activity. RESULTS: Activity of the glycosidases beta-galactosidase, alpha-mannosidase, beta-glucuronidase and beta-hexosaminidase, as well as of the peptidases cathepsin B, cathepsin D, dipeptidyl peptidase I, and tripeptidyl peptidase I, was elevated during the first 5 years of SS, and it increased further between 5 and 10 years after diagnosis. CONCLUSIONS: The elevated activities of the lysosomal enzymes in Sjögren's syndrome patients may play a role in tissue damage by accelerated breakdown of glycoproteins in lysosomes.
16163443 Corticosteroid injections reduce size of rheumatoid nodules. 2006 Feb BACKGROUND: Symptomatic rheumatoid nodules are frequently surgically treated. Injection with steroids might be an alternative treatment. PATIENTS AND METHODS: To determine whether injection with triamcinolon acetonide reduces the size of rheumatoid nodules, we randomized twenty patients with symptomatic nodules to either triamcinolon acetonide 40 mg/ml plus lidocaine 2% or lidocaine 1% (placebo). We measured the nodules before injection and 2, 4, 8, and 12 weeks after injection. Possible side effects were recorded. RESULTS: We found that the volume of the nodules injected with triamcinolon acetonide reduced significantly (p = 0.011), from 130 to 8 mm(3) (median calculated size) at 12 weeks, compared with baseline. Furthermore, at 12 weeks, the difference between the groups was significant (p = 0.03). The median size of the placebo nodules diminished as well, from 358 to 237 mm(3), but this was not significant. Pain at injection was the only side effect, equally distributed in both treatment groups. CONCLUSION: Injection with triamcinolon acetonide seems to be an alternative for surgery of rheumatoid nodules. No adverse events occurred but the limited sample does not allow definitive conclusions.
15789896 Cytomegalovirus-associated hemophagocytic syndrome in a patient with adult onset Still's d 2005 Jan Reactive hemophagocytic syndrome (HPS) is characterized by hemophagocytosis by activated histiocytes, resulting in pancytopenia and liver dysfunction. We describe a patient with adult onset Still's disease (AOSD) in whom HPS developed. An 80-year-old Japanese woman with high fever, arthralgia, skin rash, and pleuritis was admitted to our hospital for further examination. She was diagnosed with AOSD and steroid therapy was initiated. During the course of steroid therapy, a re-elevation of serum ferritin levels and a marked increase in serum transaminase were observed. Bone marrow aspiration revealed an increase in the number of histiocytes with hemophagocytosis and cytomegalovirus (CMV)-positive leukocytes were detected. At this time we diagnosed the patient as having virus-associated hemophagocytic syndrome (VAHS) and elevated levels of trasaminase and ferritin were normalized by ganciclovir treatment. Reactive HPS occurs in cases of active AOSD. However, it should be noted that HPS may be accompanied by opportunistic infections during immunosuppressive therapy requiring prompt antibiotic therapy.
16380752 [Primary Sjogren's syndrome: clinical and serological feature of a single centre]. 2005 Dec OBJECTIVE: To describe clinical and serological features of a large series of patients affected by primary Sjogren's syndrome (pSS), assessing the evolution of the disease in a long-term follow-up study. METHODS: Clinical and laboratory data of 250 patients with pSS attending our Unit for a mean follow-up period of 140 months were retrospectively collected and analysed. In all the cases the diagnosis was made according with the recent international criteria. RESULTS: Glandular involvement was almost universally present, typically as the first manifestation of the disease and a slow progression of the salivary and lachrymal dysfunction was seen during the observation period. Extraglandular involvement was mild, quite rare and delayed. The respective percentages for muscle-skeletal disease, urogenital, haematological, skin, pulmonary, gastrointestinal, neurological and renal involvement were 60%, 40%, 24%, 20%, 11%, 7%, 8% and 3%. Only 6 patients developed a lymphoma. The serological pattern of the majority of patients remained constant throughout the follow-up period. CONCLUSIONS: pSS is often a benign condition. Since some patients may develop lymphoid malignancies, clinical follow-up is recommended.
15775703 A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal 2005 We report a very rare case of autoimmune pancreatitis (AIP) associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome. The patient had an enlarged pancreas, and autoantibodies were detected in the serum. Serum IgG and IgG4 concentrations were also elevated. Endoscopic retrograde cholangiopancreatography revealed an irregular narrowing of the main pancreatic duct from the head to the body and sclerotic change in the intrapancreatic common bile duct, which later extended to the intrahepatic bile ducts. In addition, histological examination of the liver revealed lymphocytic sclerosis around the bile ducts, similar to the histology in the pancreas of AIP. Retroperitoneal tumors were diagnosed as retroperitoneal fibrosis by histological examination. Serological and functional abnormalities suggestive of Sjögren's syndrome were detected, and histological findings of the lip were compatible with Sjögren's syndrome. Immunohistochemistry of each lesion disclosed that most of the infiltrating lymphocytes were T cells with similar levels of both CD4+ and CD8+ cells. Moreover, some of the infiltrating plasma cells were positive for anti-IgG4 monoclonal antibody. These diseases were dramatically improved by steroid therapy. Although the pathophysiology of AIP is still unclear, the present case suggests a common pathophysiological mechanism for AIP, sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome.
16575363 [Sjögren syndrome in Obstetric and Gynecology: literature review]. 2006 Apr Sjogren syndrome (SS) is an immune disease characterized by a progressive degeneration of exocrine glands. It leads to dryness of mucosa and conjunctivitis. Gynecologists and obstetricians may encounter this disease in women at any age, including during pregnancy. Knowledge of the main characteristics is required for early diagnosis and multidisciplinary program. In the event of secondary Sjögren syndrome occurring during pregnancy, treatment focuses on the associated disease, mainly systemic lupus erythematosus. In primary Sjögren syndrome, pregnancy does not appear to influence disease course. However, patients with both primary and secondary Sjögren syndrome must be monitored carefully. There is a risk of neonatal lupus and congenital atrioventricular bloc associated with high morbidity and mortality. These patients should benefit from multidisciplinary care in a hospital with a neonatal intensive care unit.
16265707 Primary Sjögren's syndrome in children and adolescents: are proposed diagnostic criteria 2005 Nov OBJECTIVE: To compare the proposed criteria for the diagnosis of primary Sjögren's syndrome (pSS) in childhood to the validated American-European Consensus Group (AECG) classification criteria for pSS in adults. METHODS: Charts of 7 children with pSS seen at British Columbia's Children's Hospital (BCCH) and data on 128 children identified through Medline in the English language literature between 1963 and 2003 were reviewed for pediatric and AECG criteria for pSS. The presence of > or = 4 criteria was required to satisfy the respective classification criteria. The expert clinical opinion of pediatric rheumatologists was considered the gold standard for diagnosis. RESULTS: A total of 24/62 (39%) cases satisfied the AECG criteria; 47/62 (76%) satisfied the proposed pediatric criteria. Inclusion of recurrent parotitis increased the sensitivity of the pediatric clinical criteria. From the cases, 78/133 (59%) satisfied the pediatric oral symptom criteria; only 6/78 (8%) had xerostomia in the absence of recurrent parotitis. There was no reported case of recurrent conjunctivitis in the absence of keratoconjunctivitis sicca. We found 101/130 (78%) cases had at least one positive autoantibody test result [antinuclear antibodies (ANA), rheumatoid factor (RF), SSA, SSB]; 78/123 (63%) had autoantibodies to SSA or SSB. CONCLUSION: The AECG adult criteria for pSS should not be applied to children as the sensitivity is unacceptably low. The inclusion of recurrent parotitis increases the sensitivity of the pediatric criteria, and recurrent parotitis should alert the clinician to the possibility of pSS. The inclusion of recurrent conjunctivitis did not improve the sensitivity over the AECG ocular criteria. The addition of ANA and RF to the AECG criteria did not change the number of patients satisfying the criteria for pediatric pSS.
15743482 Multilevel examination of minor salivary gland biopsy for Sjogren's syndrome significantly 2005 The recently observed low reproducibility of focus score (FS) assessment at different section depths in a series of single minor salivary gland biopsies highlighted the need for a standardized protocol of extensive histopathological examination of such biopsies in Sjogren's syndrome. For this purpose, a cumulative focus score (cFS) was evaluated on three slides cut at 200-mum intervals from each of a series of 120 salivary biopsies. The cFS was substituted for the baseline FS in the American-European Consensus Group (AECG) criteria set for Sjogren's syndrome classification, and then test specificity and sensitivity were assessed against clinical patient re-evaluation. Test performances of the AECG classification with the original FS and the score obtained after multilevel examination were statistically compared using receiver operating characteristic (ROC) curve analysis. The diagnostic performance of AECG classification significantly improved when the cFS was entered in the AECG classification; the improvement was mostly due to increased specificity in biopsies with a baseline FS >or= 1 but <2. The assessment of a cFS obtained at three different section levels on minor salivary gland biopsies can be useful especially in biopsies with baseline FSs between 1 and 2.
16917562 Evaluation of the concordance of sialometry and salivary glands scintigraphy in dry mouth 2006 Jan INTRODUCTION: Many diagnostic tests are used to evaluate dry mouth patients, especially the ones with Sjögren's Syndrome, to whom these tests are part of classification criteria for scientific studies. AIM: Thus, the concordance between results of sialometry and salivary glands scintigraphy was evaluated; if positive, it would enable the choice of one or the other for diagnosis. PATIENTS AND METHOD: Seventy-two dry mouth patients were divided into non-Sjögren's Syndrome group, primary Sjögren's Syndrome group and secondary Sjögren's Syndrome group. The concordance among sialometry and scintigraphy results was evaluated by Kappa test. RESULTS: It was observed that their concordance was equal or near to zero. CONCLUSION: It is not possible to make a choice between these tests and both should be performed.
16491762 [Thinking and methods of treatment of Sjogren's syndrome based on syndrome differentiation 2006 Jan Sjogren's syndrome is one of clinical difficult and complicated diseases and there is no radical treatment for it at present. The authors summarize the thinking of clinical syndrome differentiation and treatment of acupuncture and moxibustion, 4 syndrome-differentiation methods and dredging the Triple Energizer channel, and needling three parts by long-term clinical practice and in combination with clinical study achievements of TCM in recent years, and put the important point of treatment on the Triple Energizer, with the points on the Triple Energizer channel as main points, combined with the points with functions of regulating functional activity of qi and regulating visceral functions, attaining better results. This provides a new method for acupuncture and moxibustion treatment of Sjogren's Syndrome.
17157574 FR177995, a novel vacuolar ATPase inhibitor, exerts not only an inhibitory effect on bone 2007 Apr There is considerable evidence that osteoclasts are involved in the pathogenesis of juxta-articular bone destruction in rheumatoid arthritis. Vacuolar ATPases (V-ATPases), which are highly expressed in the ruffled border membrane of osteoclasts, play a central role in the process of bone resorption, and V-ATPase inhibitors are effective in preventing bone destruction in several animal models of lytic bone diseases. Here, we evaluated for the first time the effects of V-ATPase inhibition in rats with adjuvant-induced arthritis (AIA) using FR177995, a novel V-ATPase inhibitor. FR177995 completely inhibited H(+) transport driven by V-ATPase, but exerted no effect on the H(+) transport activities of F- and P-ATPase, indicating that FR177995 is a specific inhibitor of V-ATPase. FR177995 acted directly on osteoclastic bone resorption and equally inhibited in vitro bone resorption stimulated by IL-1, IL-6 or PTH. In addition, FR177995 dose-dependently reduced retinoic acid-induced hypercalcemia in thyroparathyroidectomized-ovariectomized rats. When FR177995 was administered to AIA rats once a day, the loss of femoral bone mineral density was significantly improved. Moreover, indicators of cartilage damage (arthritis score and glycosaminoglycan content in the femoral condyles) and inflammation parameters (paw swelling volume, erythrocyte sedimentation rate and plasma sialic acid level) were found to be unexpectedly ameliorated. These results strongly suggest that V-ATPase may be an interesting drug target in the treatment of rheumatoid arthritis.
16570809 [Study on mechanism underlying the treatment of rheumatoid arthritis by Keshiling]. 2006 Jan OBJECTIVE: To explore the mechanism underlying the treatment of rheumatoid arthritis by Keshiling (KSL) in the rats model of FCA-induced arthritis. METHOD: The experimental arthritis was induced by FCA in the rats. The content of PGE2 in the inflammatory swelling toes was evaluated by ultraviolet spectrophotometric method. The ConA and LPS-induced lymphocytes proliferation and the production of interleukin-2 (IL-2) secreted by thymus were determined by MTT assay. RESULT: Results showed that the increases of lymphocyte proliferation and IL-2 production in AIA rats could be inhibited by KSL at the concentrations of 540 and 270 mg x kg(-1) in vivo and vitro. KSL at the same doses decreased the contents of PGE2 in inflammatory swelling toes, and the decreased A values were 25.6,16.1, 10.0 (A x 10(3)), respectively. After administration of KSL in vivo at 540 and 270 mg x kg(-1) the T lymphocyte proliferation were attenuated by 32.1% and 31.0%, and the production of IL-2 was inhibited by 17.5% and 14.0% respectively. While the inhibitory rates of T lymphocyte proliferation were reduced by 39.0% and 22.1% and the production of IL-2 was diminished by 27.3% and 18.2% respectively following the administration of KSL in vitro. CONCLUSION: KSL possesses the anti-inflammation function.
16287587 Early undifferentiated arthritis. 2005 Nov Patients who cannot be classified as having a well-defined arthropathy typically are labeled as having undifferentiated arthritis. Some patients develop sufficient features to permit classification, whereas others remain undifferentiated, but with persistent joint inflammation, functional disability, and development of radiographic damage. Identifying the subset of patients destined to develop rheumatoid arthritis, spondyloarthropathy, or a more severe and persistent form of undifferentiated arthritis and choosing appropriate treatment strategies remain challenges for clinicians. Numerous investigative strategies are available with which to characterize undifferentiated arthritis and assess prognosis. This article discusses the characteristics of undifferentiated arthritis at presentation and the investigative strategies that can be used to predict prognosis and outcome early in the disease course. Therapeutic strategies also are explored.
15820372 Measurement of health status, functional status, and quality of life in children with juve 2005 Apr Several groups have undertaken research on health status, functional status, and quality of life in the pediatric rheumatic diseases, particularly juvenile idiopathic arthritis (JIA) and juvenile rheumatoid arthritis. This article highlights the principles involved in this type of measurement, discusses the measures that have been developed for JIA, and describes the outcomes determined from recent retrospective and prospective longitudinal outcome studies. These studies suggest that although there has been improvement in overall outcomes, significant numbers of individuals persist with active disease into adulthood and have significant damage, reduced functional ability, and disability.
15936744 Ncf1 (p47phox) polymorphism determines oxidative burst and the severity of arthritis in ra 2005 Feb Identifying genes that regulate polygenic diseases influenced by the environment such as rheumatoid arthritis (RA), has so far proven to be difficult. By using an alternative approach, i.e., linkage analysis using relevant animal models we succeeded in finding the Ncf1 gene residing in the Pia4 quantitative trait locus to be responsible for the severity of pristane induced arthritis in rats. The influence of another mutation in the mouse Ncf1 gene showed the same association between decreased oxidative burst and enhanced arthritis. In this case the mutation affected a splice site giving a non-detectable oxidative burst response and enhanced collagen induced arthritis as well as myelin oligodendrocyte protein induced experimental autoimmune encephalomyelitis. These findings open up new possibilities for new treatments for autoimmune diseases, i.e., RA, targeting the NADPH oxidase pathway.