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  • SAGE Publications  (2)
  • 1
    Online Resource
    Online Resource
    SAGE Publications ; 2016
    In:  Journal of Scleroderma and Related Disorders Vol. 1, No. 2 ( 2016-05), p. 234-240
    In: Journal of Scleroderma and Related Disorders, SAGE Publications, Vol. 1, No. 2 ( 2016-05), p. 234-240
    Abstract: Randomized controlled trials have shown that cyclophosphamide (CYC) was an option in systemic sclerosis-associated interstitial lung disease (SSc-ILD). The observed improvement disappeared after CYC was stopped suggesting that a maintenance regimen was mandatory. Immunosuppressants were suggested to be more effective in patients with worsening (i.e., with worsening of dyspnea and/or pulmonary functional tests) SSc-ILD. We aimed to assess the efficacy of mycophenolate mofetil (MMF) as a maintenance regimen after CYC in worsening SSc-ILD. Methods All patients (n = 20) with worsening SSc-ILD were retrospectively included. Treatment consisted of 6 to 12 monthly pulses of CYC followed by MMF and response was assessed by the evolution of the forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) during follow-up. Results At the end of CYC pulses, SSc-ILD had improved in 7 (35%) patients, stabilized in 10 (50%) (i.e., 85% of responders) and worsened in 3 (15%) when compared to baseline. After 6 months of MMF, 70% were still responders while 30% had worsened when compared to baseline (i.e., before CYC). After 12 months on MMF, 55% were responders and 45% had worsened when compared to baseline. Evolution of the FVC slope significantly improved on CYC as well as on MMF. Conclusions A strategy combining IV CYC followed by maintenance MMF for worsening SSc-ILD was associated with stabilization or improvement of pulmonary function tests in only 55% of patients after 12 months of MMF. This suggests that careful monitoring for worsening is mandatory during MMF maintenance and that improvement in managing worsening SSc-ILD is still needed.
    Type of Medium: Online Resource
    ISSN: 2397-1983 , 2397-1991
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2016
    detail.hit.zdb_id: 2964332-6
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  • 2
    In: Vascular Medicine, SAGE Publications, Vol. 15, No. 6 ( 2010-12), p. 451-459
    Abstract: Inferior vena cava agenesis (IVCA) is a rare condition, found in almost 5% of patients under 30 years old with unprovoked deep venous thrombosis (DVT). We describe 10 consecutive patients with IVCA-associated DVT and conducted an extensive literature review to investigate the typical spectrum of IVCA-associated DVT. Among our patients (eight men and two women; mean age, 25 ± 4.5 years), DVT followed intense and unusual (major) physical activity for eight of them. DVT was bilateral in six patients and unilateral in four. Ultrasonography was unable to detect IVCA, which was visualized by computed-tomography scans for seven patients, and magnetic resonance imaging and angiography for 10. Hereditary thrombophilia screening, to detect factor V Leiden or prothrombin gene heterozygosity (G20210A mutation), was positive for only two patients. Wearing elastic stockings and taking an indefinite or long-term vitamin K antagonist were prescribed for all 10 patients and nine complied with the latter. To date, 62 patients with IVCA-associated DVT have been reported in the English literature. Analysis of them and our patients yielded a typical spectrum of IVCA-associated DVT characteristics: IVCA occurs in young adults, particularly males, and is revealed by proximal DVT following major physical exertion. All were treated with a prolonged vitamin K antagonist and advised to wear elastic stockings. No precise duration of anticoagulation has been established.
    Type of Medium: Online Resource
    ISSN: 1358-863X , 1477-0377
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2010
    detail.hit.zdb_id: 2027562-6
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