bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deficiency of the β subunit of the eighth component of complement presenting as arthritis and exanthem
Authors: Wulffraat N. M. , Sanders E. A. M. , Fijen C. A. P. , Hannema A. , Kuis W. , Zegers B. J. M. 
Published: February 2007

AbstractA 13‐year‐old boy presented with juvenile chronic arthritis of 6 months' duration. Antinuclear antibodies, anti–double‐stranded DNA antibodies, and rheumatoid factor were not detected. Western blotting showed a deficiency of the β subunit of the eighth component of complement. The same deficiency was present in the patient's sister. C8β deficiency is usually detected in individuals who survive meningococcal disease. There was no such history in this family. Juvenile chronic arthritis has not previously been described in patients with C8β deficiency.
ELEVATED SERUM LEVELS OF INTERLEUKIN‐1 RECEPTOR ANTAGONIST IN POLYMYOSITIS/DERMATOMYOSITIS. A Biologic Marker of Disease Activity with a Possible Role in the Lack of Acute‐Phase Protein Response
Authors: Gabay Cem , Gay‐Croisier Fabienne , Roux‐Lombard Pascale , Meyer Olivier , Maineti C. , Guerne Pierre‐André , Vischer Thomas , Dayer Jean‐Michel 
Published: February 2007

AbstractObjective. To determine whether the lack of acute‐phase protein (APP) response in numerous patients with polymyositis/dermatomyositis (PM/DM) is related to an imbalance between cytokines and cytokine inhibitors.Methods. Levels of C‐reactive protein (CRP), interleukin‐1β (IL‐1β), tumor necrosis factor α (TNFα), IL‐6, IL‐1 receptor antagonist (IL‐1ra), TNF soluble receptor 55 kd (sTNFR 55 kd), and sTNFR 75 kd were tested in the serum of 15 patients with PM/DM, 14 patients with spondylarthropathies (SPA), and 12 healthy blood donors. Serum IL‐1β, TNFα, IL‐6, IL‐1ra, sTNFR were measured by specific immunoassays.Results. Serum levels of CRP were lower in PM/DM patients than in SPA patients. Normal or slightly elevated CRP values were found in 10 of the 15 PM/DM patients, 7 of whom had active myositis. Serum IL‐6 levels were significantly higher in SPA patients than in PM/DM patients, whereas serum IL‐1ra and sTNFR levels were significantly higher in PM/DM than in SPA patients. IL‐1ra levels were particularly elevated in patients with active myositis and decreased in response to treatment.Conclusion. These differences in cytokine levels, particularly IL‐1ra, between PM/DM and SPA patients are indicative of distinct pathogenic mechanisms. High levels of IL‐1ra may account for the weak APP response in some PM/DM patients. Our results suggest that measurement of IL‐1ra, together with clinical examination, may provide useful information for the followup of PM/DM patients.
Methotrexate concentrations in synovial membrane and trabecular and cortical bone in rheumatoid arthritis patients
Authors: Bologna Christophe , Edno Leila , Anaya Juan‐Manuel , Canovas Francois , Berghe Marc Vanden , Jorgensen Christian , Galtier Marc , Combe Bernard , Bressolle Francoise , Sany Jacques 
Published: February 2007

AbstractObjective. To determine methotrexate (MTX) concentrations in the synovial membrane (SM) and cortical and trabecular bone of rheumatoid arthritis (RA) patients.Methods. Ten RA patients (9 women, 1 man; mean ± SD age 49.2 ± 10.6, mean disease duration 13.2 ± 9.9 years) undergoing surgical procedures for rheumatoid articular lesions participated in this study. Mean ± SD MTX treatment duration was 26.4 ± 21.3 months. The day preceding surgery, 10 mg of MTX was administered intramuscularly. During surgery, a mean ± SD of 19.7 ± 2.6 hours after MTX administration, SM, bone fragments, and blood were collected simultaneously. MTX was assayed by fluorescence polarization immunoassay in plasma and tissues.Results. The mean ± SD plasma concentration was 0.0252 ± 0.01 nmoles/ml at the time of tissue sampling. The mean MTX concentration in SM was 0.285 ± 0.159 nmoles/gm. The mean MTX concentrations in trabecular and cortical bone were 0.292 ± 0.164 and 0.286 ± 0.126 nmoles/gm, respectively.Conclusion. After intramuscular administration, high MTX concentrations are found in SM and cortical and trabecular bone of RA patients.
Antibodies to histone (h2a–h2b)–dna complexes in the absence of antibodies to double‐stranded dna or to (h2a–h2b) complexes are more sensitive and specific for scleroderma‐related disorders than for lupus
Authors: Wallace Daniel J. , Lin Hun‐Chi , Shen Guo Qiu , Peter James B. 
Published: February 2007

AbstractObjective. To assess the role of antibodies to histones H2A, H2B, and anti–double‐stranded DNA which form (H2A–H2B)–DNA complexes in patients with scleroderma‐related disorders.Methods. Antihistone antibodies were measured, by enzyme immunoassay, in 26 patients with scleroderma‐related disorders, 100 patients with systemic lupus erythematosus (SLE), and 24 patients with rheumatoid arthritis.Results. Antibodies to histone (H2A–H2B)–DNA complex were more commonly seen in patients with scleroderma‐related disorders than in those with SLE (P < 0.0001).Conclusion. Scleroderma‐related disorders should be included among conditions in which various types of antihistone antibodies are produced. A hypothesis to account for this finding is discussed.
Changes in cartilage composition and physical properties due to stromelysin degradation
Authors: Bonassar Lawrence J. , Frank Eliot H. , Murray Jane C. , Paguio Claribel G. , Moore Vernon L. , Lark Michael W. , Sandy John D. , Wu Jiann‐Jiu , Eyre David R. , Grodzinsky Alan J. 
Published: February 2007

AbstractObjective. To determine the effects of stromelysin treatment on biochemical, histologic, and swelling characteristics of intact cartilage explants and to correlate these effects with changes in the functional physical properties of the tissue.Methods. Bovine articular cartilage explants were cultured for up to 3 days in the presence or absence of recombinant human stromelysin (SLN). Damage to matrix proteoglycans and collagens was assessed and characterized by N‐terminal sequencing and Western blot analysis, respectively. Explants were mechanically tested to assess the ability of the tissue to withstand cyclic and static compressive loads.Results. Treatment with SLN resulted in a time‐and dose‐dependent loss of proteoglycans from cartilage explants, with significant loss seen after 3 days of exposure to 20 nM SLN. Histology indicated that initial loss of proteoglycans occurred in regions near the tissue surface and proceeded inward with increasing time of SLN exposure. SLN treatment resulted in degradation of matrix collagen types IX and II, and a concomitant increase in tissue swelling. This matrix degradation resulted in severe alterations in functional physical properties of the tissue, including compressive stiffness. The initial, focal loss of proteoglycans that resulted from SLN treatment was most accurately detected with highfrequency streaming potential measurements.Conclusion. Exposure of intact cartilage to SLN caused specific, molecular‐level degradation of matrix molecules, which resulted in changes in the swelling behavior and marked deterioration of functional physical properties of the tissue.
Acceleration of experimental lapine osteoarthritis by calcium pyrophosphate microcrystalline synovitis
Authors: Fam Adel G. , Morava‐Protzner Isabella , Purcell Carrie , Young Beverly D. , Bunting Peter S. , Lewis Anthony J. 
Published: February 2007

AbstractObjective. To investigate the effects of chronic calcium pyrophosphate dihydrate (CPPD) synovitis on the development of osteoarthritic (OA) lesions in an animal model.Methods. OA was induced in the right knees of 30 male New Zealand white rabbits by partial lateral meniscectomy and section of the fibular collateral and sesamoid ligaments (PLM/LS), followed by 8 weekly intraarticular (IA) injections of 1 mg (low‐dose) or 10 mg (high‐dose) of CPPD crystals in 3 sets of experiments (10 rabbits each). The contralateral left knees served as controls: experiment 1 PLM/LS alone, experiment 2 8 weekly IA injections of CPPD crystals alone, and experiment 3 sham surgery plus 8 weekly IA injections of CPPD crystals.Results. At 8 weeks, repeated IA injections of low‐dose and high‐dose CPPD crystals into meniscectomized right knees resulted in more severe OA than in meniscectomized but noninjected left knees (experiment 1) (P = 0.003 and P = 0.001, respectively). One‐fourth of the meniscectomized knees (11 of 40), both CPPD‐injected and noninjected, showed embedded synovial cartilage shards.Conclusion. The data demonstrate a worsening effect of chronic CPPD crystal‐induced synovitis on experimental OA produced in the rabbit knees by PLM/LS, and support a possible role for CPPD microcrystalline inflammation in the progression of OA lesions in clinical CPPD crystal deposition disease.
The independence and stability of socioeconomic predictors of morbidity in systemic lupus erythematosus
Authors: Karlson Elizabeth W. , Daltroy Lawren H. , Lew Robert A. , Wright Elizabeth A. , Partridge Alison J. , Roberts W. Neal , Stern Steven H. , Straaton Karin V. , Wacholtz Mary C. , Grosflam Jodi M. , Liang Matthew H. 
Published: February 2007

AbstractObjective. We studied the relationship between systemic lupus erythematosus (SLE) morbidity and socioeconomic status (SES) at 5 centers.Methods. Ninety‐nine patients who met American College of Rheumatology criteria for SLE were randomly sampled at each center, balancing by race and insurance status. Subjects were interviewed for current and past SES factors, such as insurance, occupation, employment, education, and income. SLE disease activity was measured by the SLE Activity Measure (SLAM).Result. Higher education, private insurance/Medicare, and higher income were associated with less disease activity at diagnosis. Controlling for SES, race, and center, the best predictors of less active disease at diagnosis were private insurance/Medicare (P = 0.002) and higher education (P = 0.007). From the time of diagnosis to the study visit (mean 3.5 years), insurance, income, and employment status changed for a significant number of subjects (37%, 16%, and 21%, respectively).Conclusion. Private insurance or Medicare and higher education are associated with less active disease at diagnosis of SLE. Health insurance, income, and employment status are unstable measures of socio‐economic status and may explain the variability in conclusions of previous studies on the role of SES in SLE.
The cost‐effectiveness of liver biopsy in rheumatoid arthritis patients treated with methotrexate
Authors: Bergquist Steven R. , Felson David T. , Prashker Mark J. , Freedberg Kenneth A. 
Published: February 2007

AbstractObjective. To assess the cost‐effectiveness of liver biopsy in monitoring rheumatoid arthritis (RA) patients for methotrexate (MTX)–induced cirrhosis.Methods. A decision analytic model was used to compare a strategy of no biopsy versus strategies of biopsy after 5 years or 10 years of MTX treatment.Results. Biopsy after 5 years of MTX treatment had a cost‐effectiveness ratio of $1,891,830 per year of life saved, while biopsy after 10 years of treatment had a cost‐effectiveness ratio of $52,374 per year of life saved. Sensitivity analyses revealed that the cost‐effectiveness of biopsy was most dependent on the probability of cirrhosis.Conclusion. Liver biopsy to monitor for MTX‐induced cirrhosis in RA patients is not cost effective after 5 years of treatment, and even biopsy after 10 years has a high cost.
Guidelines for clinical trials in systemic sclerosis (scleroderma)
Authors: White Barbara , Bauer Eugene A. , Goldsmith Lowell A. , Hochberg Marc C. , Katz Linda M. , Korn Joseph H. , Lachenbruch Peter A. , Carwile Leroy E. , Mitrane Margaret P. , Paulus Harold E. , Postlethwaite Arnold E. , Steen Virginia D. 
Published: February 2007

AbstractObjective.To develop guidelines for therapeutic trials designed to improve the overall course of systemic sclerosis (SSc), that is, to reduce the development of significant organ damage or death.Methods.A committee developed general guidelines for patient inclusion and exclusion criteria, randomization, blinding of patients and physicians, controls, duration of the trial, investigator training, responses, samples size, study dropouts, statistical analyses, data management, and safety monitoring. Delphi and nominal group techniques were used.Results.Briefly, patients with diffuse cutaneous SSc of less than 24 months' duration should be included because they are at greatest risk for the development of severe organ damage and death. Patients should be excluded if they have other connective tissue diseases, SSc‐like illnesses related to exposures or ingestions, severe existing internal organ damage, an unacceptable risk of side effects, or concurrent therapies that might independently influence the outcome. Randomized, double‐blind, placebo‐controlled trials are preferred. The treatment and followup period must be long enough to permit observation of any disease modification, which is likely to require 18–36 months, unless an extraordinarily effective therapy is identified. Responses selected should be quantitative, consistently and accurately reflect activity of SSc in major target organs (not solely the skin), be sensitive to change, and be standardized, with limited variability. An example of a set of responses is given. Surrogate responses are desirable, but none have been validated as correlating with organ damage.Conclusion.Guidelines have been established for trials of disease‐modifying interventions in SSc. These guidelines will need to be altered as additional information becomes available. Any given protocol will be individualized based on the nature of the intervention and objectives of the study. Nonetheless, each study team should develop a protocol that meets the spirit of these guidelines.
Correlation between infection and the onset of the giant cell (temporal) arteritis syndrome
Authors: Russo Michael G. , Waxman Jack , Abdoh Ahmed A. , Serebro Leonard H. 
Published: February 2007

AbstractObjective. To assess for a correlation between infection and the onset of the giant cell (temporal) arteritis (GCA) syndrome.Methods. A matched case‐control study design was used. Records of 100 patients with biopsy‐proven GCA and 100 patients undergoing corrective surgery for hip fracture who did not have GCA were retrospectively reviewed. Non‐GCA patients were sex‐matched with GCA patients and were as old or older in age. The review period for GCA patients was up to 4 months before and during the occurrence of symptoms (median 2 months), and for non‐GCA patients, it was up to 7 months before hip fracture. The prevalence of infection was compared using matched‐pairs odds ratios and their 95% confidence intervals.Results. Infections were 3 times more likely to occur in GCA patients than in non‐GCA patients (P < 0.05).Conclusion. A correlation between the occurrence of infection and the onset of GCA is strongly suggested. We speculate that infection may act as a trigger mechanism in the pathogenesis of this syndrome.
Increased insulin‐like growth factor 1 production by human osteoarthritic chondrocytes is not dependent on growth hormone action
Authors: Doré Sylvain , Abribat Thierry , Rousseau Nicole , Brazeau Paul , Tardif Ginette , Dibattista John Anthony , Cloutier Jean‐Marie , Pelletier Jean‐Pierre , Martel‐Pelletier Johanne 
Published: February 2007

AbstractObjective. To investigate insulin‐like growth factor 1 (IGF‐1) production in normal and osteoarthritis (OA) chondrocytes and to further examine the role of growth hormone (GH) in adult human cartilage and, in particular, in diseased tissue.Methods. IGF‐1 production was measured with a radioimmunoassay. Binding assay, Northern blot, and reverse transcriptase polymerase chain reaction (RT‐PCR) techniques were used for GH receptor (GHR) detection. The biological response to GH was estimated via IGF‐1 production.Results. We observed that basal levels of IGF‐1 production were significantly higher in OA chondrocytes than in normal cells (P < 0.005). Adult human chondrocytes, however, were unresponsive to GH stimulation with regard to IGF‐1 production, as shown in dose‐response (0–1,000 ng/ml) and time‐course (days 1–8) studies. In addition, no specific 125I‐GH binding was detected in either cell type. Northern blot analysis revealed a 5.5‐kb GHR messenger RNA (mRNA) species, but semiquantitative RT‐PCR revealed no difference in GHR mRNA expression by normal and OA chondrocytes.Conclusion. This study indicates that the elevated synthesis of IGF‐1 by adult human OA chondrocytes occurs through a GH/GHR‐independent mechanism, suggesting that other factors are capable of controlling local IGF‐1 production in these cells.
Excessive function of peripheral blood neutrophils from patients with behcet's disease and from hla‐b51 transgenic mice
Authors: Takeno Mitsuhiro , Kariyone Ai , Yamashita Naomi , Takiguchi Masafumi , Mizushima Yutaka , Kaneoka Hidetoshi , Sakane Tsuyoshi 
Published: February 2007

AbstractObjective. To elucidate the role played by HLA‐B51 in the neutrophil hyperfunction of Behcet's disease, we determined the superoxide production by purified peripheral blood neutrophils from Behcet's disease patients, from HLA‐B51 positive healthy individuals, and from HLA‐B51 transgenic mice.Methods. Neutrophil function was evaluated by flow cytometric analysis, detecting the conversion of 2′,7′‐dichlorofluorescin diacetate into dichlorofluorescein, induced by superoxide in the neutrophils.Results. A significant correlation between the neutrophil hyperfunction and the possession of HLA‐B51 phenotype, regardless of the presence of the disease, was observed in humans. FMLP‐stimulated neutrophils (without in vitro priming) from HLA‐B51 transgenic mice, but not those from HLA‐B35 transgenic mice or from nontransgenic mice, produced substantial amounts of superoxide.Conclusion. The HLA‐B51 molecule itself may be responsible, at least in part, for neutrophil hyper‐function in Behcst's disease.
Demonstration of granzyme A and perforin messenger RNA in the synovium of patients with rheumatoid arthritis
Authors: MÜLler‐Ladner Ulf , Kriegsmann JÖRg , Tschopp JÜRg , Gay Renate E. , Gay Steffen 
Published: February 2007

AbstractObjective. To examine the gene expression of 2 highly specific markers of cytotoxic T lymphocyte (CTL) activation, the serine protease granzyme A and the poreforming protein perforin, in synovial tissue of patients with rheumatoid arthritis (RA), and to compare the findings with those in osteoarthritis (OA) synovial tissue.Methods. Snap‐frozen synovial tissue specimens from 9 patients with RA and 5 patients with OA were examined. The number of CTL that expressed granzyme A or perforin messenger RNA was determined by in situ hybridization using nonradioactive riboprobes for granzyme A and perforin, and by a novel in situ reverse transcriptase technique. The signals were visualized by an immunogold–silver immunohistochemistry technique and compared with immunohistochemical labeling of T and B cells. Additional double‐labeling was achieved using anti–type IV collagen, anti‐macrophage (anti‐CD68), anti–T lymphocyte (anti‐CD45RO), anti–B lymphocyte (anti‐CD20), and anti–natural killer cell (anti‐CD56) antibodies in an alkaline phosphatase–anti–alkaline phosphatase assay.Results. Granzyme A and perforin messenger RNA (mRNA) was observed in CTL in synovial specimens from all of the RA patients, whereas in specimens from OA patients only a few, single cells with a positive mRNA signal for these molecules could be detected. In the RA specimens, the number of lymphocytes showing a positive mRNA signal for granzyme A or perforin varied from 10% to 50%, reflecting the recent findings of other investigators studying synovial fluid.Conclusion. Our results demonstrate that gene expression of at least 2 CTL products, granzyme A and perforin, is up‐regulated in the synovium of patients with RA compared with that in the synovium of patients with OA. These molecules presumably play an important role not only in lymphocyte‐mediated cytotoxicity, but also in facilitating the migration of blood‐born mononuclear cells through the vascular basement membrane into the rheumatoid synovium.
Impaired polyclonal t cell cytolytic activity
Authors: Stohl William 
Published: February 2007

AbstractObjective. To determine whether impaired generation of polyclonal T cell cytolytic activity is over‐represented in systemic lupus erythematosus (SLE) compared with other rheumatologic diseases and whether such impaired generation of cytolytic activity waxes and wanes with disease activity and/or changes in medications.Methods. Peripheral blood mononuclear cells from 84 SLE patients, 55 rheumatologic disease (RD) controls, and 44 normal subjects were stimulated with anti‐CD3 monoclonal antibody, maintained in interleukin‐2, and assayed for cytolytic activity against 51Cr‐labeled Daudi target cells.Results. Generation of cytolytic activity was significantly lower in SLE patients than in either RD or normal controls. Abnormal cytolytic responses in SLE could not be attributed to the patient's sex, race, age, disease activity, or antirheumatic medications (including corticosteroids and cytotoxics), although both SLE and RD patients taking azathioprine (AZA) manifested lower responses than did corresponding patients not taking AZA. Abnormal cytolytic activity reflected, in large measure, impaired cytolytic activity of CD8+ T cells. No significant difference in the generation of cytolytic activity between RD and normal controls was detected.Conclusion. Impaired generation of polyclonal T cell cytolytic activity may be a predisposing factor in the development of SLE.
Cross‐linked elastin and collagen degradation products in the urine of patients with scleroderma
Authors: Stone Phillip J. , Korn Joseph H. , North Heather , Lally Edward V. , Miller Laurie C. , Tucker Lori B. , Strongwater Steven , Snider Gordon L. , Franzblau Carl 
Published: February 2007

AbstractObjective. To measure the urinary excretion of specific cross‐link amino acid markers for mature elastin (desmosine [DES] and isodesmosine [IDES]) and fibrillar collagen (hydroxylysylpyridinoline [HP] and lysylpyridinoline [LP]) in systemic sclerosis (SSc) patients and healthy controls.Methods. Urine specimens from 20 patients with SSc and 22 controls were assessed for DES, IDES, HP, and LP using high performance liquid chromatography and ultraviolet absorption spectroscopy, in combination with an isotope dilution technique in which the urine specimen was spiked with isotopically labeled cross‐link amino acids.Results. Mean ± SD levels of urinary DES and IDES were elevated in SSc patients by 2–3‐fold, and urinary HP and LP by 3–4‐fold, compared with controls (DES 21.0 ± 9.4 versus 7.5 ± 1.4 μg/gm creatinine; HP 109.0 ± 72.9 versus 24.9 ± 5.7 nmoles/mmole creatinine). Nineteen of the 20 SSc patients had urinary DES and HP values that were >3 SD above the control mean. A significant elevation in the HP:LP ratio in SSc patients as compared with controls (mean ± SD 6.9 ± 1.5 versus 5.5 ± 1.3) indicated a soft tissue origin for much of the increased HP.Conclusion. Patients with SSc have higher levels of urinary cross‐link amino acids specific for the degradation of mature collagen and elastin. These markers distinguish most SSc patients from healthy controls.
Critical appraisal of continuing medical education in the rheumatic diseases for primary care physicians
Authors: Glazier Richard , Buchbinder Rachelle , Bell Mary 
Published: February 2007

AbstractObjective. To critically appraise evidence of the effectiveness of continuing medical education (CME) in rheumatic diseases for primary care physicians.Methods. Three physicians independently applied preset criteria to evaluated CME interventions published between January 1966 and August 1993.Results. Eight of 166 articles identified were critically appraised, 7 of which had positive results. Marked heterogeneity in educational interventions, evaluative methods, and outcomes was noted.Conclusion. Despite generally positive results, weak methodology precludes drawing firm conclusions about the effectiveness of CME in rheumatic diseases.