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Fulminant onset of cerebral immunocomplex vasculitis as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE)

Department of Neurology, Ruprecht Karls University Heidelberg, Heidelberg, Germany Timolaos.Rizos{at}med.uni-heidelberg.de

M Siegelin

Department of Neuropathology, Ruprecht Karls University Heidelberg, Heidelberg, Germany

S Hähnel

Department of Neuroradiology, Ruprecht Karls University Heidelberg, Heidelberg, Germany

B Storch-Hagenlocher

Department of Neurology, Ruprecht Karls University Heidelberg, Heidelberg, Germany

A Hug

Department of Neurology, Ruprecht Karls University Heidelberg, Heidelberg, Germany

Fulminant onset of neuropsychiatric symptoms as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE) is rare and diagnosis is difficult if only one organ is involved. Here, we report the case of a previously healthy woman who presented with a clinical syndrome most compatible with acute onset of NPSLE. However, American College of Rheumatology (ACR) criteria were not sufficiently met. Brain biopsy showed an autoimmune complex vasculitis consistent with central nervous system (CNS) lupus. Because the prognosis of SLE-related CNS involvement is poor, aggressive immunosuppressive treatment was initiated using methylprednisolone in combination with cyclophosphamide.

Key Words: neuropsychiatric systemic lupus erythematosus • systemic lupus erythematosus • vasculitis • brain biopsy • neurological ICU

Lupus, Vol. 18, No. 4, 361-363 (2009)
DOI: 10.1177/0961203308097448


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