Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions.

Catastrophic primary central nervous system vasculitis / Salvarani, Carlo; Brown, Robert D; Morris, Jonathan M; Huston, John; Hunder, Gene G.. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 32:(2014), pp. S3-S4.

Catastrophic primary central nervous system vasculitis

SALVARANI, CARLO;
2014

Abstract

Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions.
2014
32
S3
S4
Catastrophic primary central nervous system vasculitis / Salvarani, Carlo; Brown, Robert D; Morris, Jonathan M; Huston, John; Hunder, Gene G.. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 32:(2014), pp. S3-S4.
Salvarani, Carlo; Brown, Robert D; Morris, Jonathan M; Huston, John; Hunder, Gene G.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1078839
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