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07-11-2017 | Vasculitis | Review | Article

Therapeutic Management of CNS Vasculitis

Current Treatment Options in Rheumatology

Authors: MD Hiromichi Tamaki, MD Rula A. Hajj-Ali

Publisher: Springer International Publishing


Purpose of review The management of primary angiitis of the central nervous system (PACNS) can be very challenging due to multitude of reasons. Despite the many challenges, a tremendous progress has been made at many levels in PACNS. Here we review how to approach PACNS including our own experience at the specialized center.
Recent findings Now, most of experts agree that glucocorticoids are the foundation of the initial treatment for PACNS with or without an additional cytotoxic agent such as cyclophosphamide depending on severity of the disease. Recent studies suggested maintenance therapy likely reduces a risk of relapse and there might be disease subcategories with different prognostic implications.
Summary There are still significant unmet needs in this arena. To advance the field, it is necessary to establish consensus on disease subcategories and research outcome tools for PACNS. Additional deeper molecular understanding of PACNS as well as clinical therapeutic research will not be fruitful without them and a consorted international effort is crucial.

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