Arthritis and rheumatism
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Arthritis and rheumatism · Sep 2003
Case ReportsFavorable preliminary experience with etanercept in two patients with the hyperimmunoglobulinemia D and periodic fever syndrome.
The hyperimmunoglobulinemia D and periodic fever syndrome (HIDS; MIM 260920) is caused by recessive mutations in the mevalonate kinase gene (MVK), which encodes an enzyme involved in cholesterol and nonsterol isoprenoid biosynthesis. HIDS is characterized by persistently elevated polyclonal IgD and recurrent febrile episodes. Although abnormalities in tumor necrosis factor alpha (TNF alpha) are not the primary cause of HIDS, plasma TNF alpha levels are elevated in HIDS patients during attacks and thus may be a therapeutic target. This study assessed the effects of etanercept, a soluble p75 TNF alpha receptor-Fc fusion protein, in 2 patients with HIDS. ⋯ Our favorable experience with etanercept for the treatment of HIDS suggests that further investigation of this therapy is warranted.
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Arthritis and rheumatism · Sep 2003
Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes.
To investigate the prevalence of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among outpatients presenting with recurrent fevers and clinical features consistent with TRAPS. ⋯ The presence of TNFRSF1A shedding defects and low sTNFRSF1A levels in 3 families without a TNFRSF1A mutation indicates that the genetic basis among patients with "TRAPS-like" features is heterogeneous. TNFRSF1A mutations are not commonly associated with nonfamilial recurrent fevers of unknown etiology.
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Arthritis and rheumatism · Aug 2003
Predicting the onset of forearm pain: a prospective study across 12 occupational groups.
To determine, among workers free of forearm pain, the role of mechanical and psychosocial factors in predicting future onset. ⋯ Along with repetitive movements of the arms and wrists, mechanical postural factors and psychosocial factors also are important risk factors for onset of forearm pain. Our study emphasizes the multifactorial nature of risks for onset of forearm pain, and provides leads as to possible mechanisms for prevention.
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Arthritis and rheumatism · Aug 2003
Physical function and health-related quality of life of Spanish patients with ankylosing spondylitis.
To determine the physical function and the quality of life (QOL) of Spanish patients with ankylosing spondylitis (AS), and to study the reliability of the Spanish version of the Bath Ankylosing Spondylitis Functional Index (BASFI). ⋯ Physical function and QOL are deteriorated in AS. The physical domain is more impaired than the mental one. The SF-36 and the health profile of the EuroQol may be used as generic instruments to measure health-related QOL. Spanish BASFI index is a reliable instrument.
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Arthritis and rheumatism · Aug 2003
Randomized Controlled Trial Multicenter Study Clinical TrialLimited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial.
To report baseline data on 180 patients with Wegener's granulomatosis (WG) enrolled in the WG Etanercept Trial (WGET), and to examine demographic and clinical differences between patients with limited disease and those with severe disease. ⋯ There are significant differences between patients with limited WG and those with severe WG with regard to sex, age, the likelihood of recurrent disease, the risk of damage in certain organ systems, and, possibly, etiologic factors. These differences (and perhaps other differences that are currently unrecognized) in patient subsets may have implications for mechanisms of pathogenesis, prognosis, response to treatment, and the design of future clinical investigations.