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  • 1.
    Berntsson, Shala Ghaderi
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Katsarogiannis, Evangelos
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Lourenco, Filipa
    Ctr Hosp Lisboa Cent, Hosp Curry Cabral, Serv Med 7 2, Unidade Doencas Autoimunes, Lisbon, Portugal..
    Moraes-Fontes, Maria Francisca
    Ctr Hosp Lisboa Cent, Hosp Curry Cabral, Serv Med 7 2, Unidade Doencas Autoimunes, Lisbon, Portugal..
    Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology2016In: Case Reports in Neurology, ISSN 1662-680X, E-ISSN 1662-680X, Vol. 8, no 1, p. 59-65Article in journal (Refereed)
    Abstract [en]

    Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

  • 2. Landtblom, A. -M
    et al.
    Katsarogiannis, Evangelos
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Kristoffersson, A.
    Linkoping Univ, Motala Gen Hosp, Motala, Sweden.
    Berntsson, S. Ghaderi
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Semnic, R.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Radiology.
    Boström, I.
    Linkoping Univ, Neurol, Linkoping, Sweden.
    Challenges in diagnosing OCB-negative MS - the importance of imaging2018In: Multiple Sclerosis, ISSN 1352-4585, E-ISSN 1477-0970, Vol. 24, p. 743-743Article in journal (Other academic)
  • 3.
    Lourenco, F.
    et al.
    Hosp Curry Cabral, Autoimmune Dis Unit, CHLC, Lisbon, Portugal..
    Katsarogiannis, Evangelos
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Berntsson, Shala
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Moraes-Fontes, M. F.
    Hosp Curry Cabral, Autoimmune Dis Unit, CHLC, Lisbon, Portugal..
    Systemic Lupus Erythematous and Progressive Multifocal Leukoencephalopathy: focus on lymphopenia2015In: Clinical and Experimental Rheumatology, ISSN 0392-856X, E-ISSN 1593-098X, Vol. 33, no 3, p. S33-S33Article in journal (Other academic)
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