Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system

Case report and review of the literature

A. Ghinoi, G. Zuccoli, N. Pipitone, C. Salvarani

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Objectives: To report a case of biopsyproven, ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and to review the relevant literature. Methods: Descriptive case report of one patient with AAV-related CNS vasculitis and review of the relevant literature (PubMed search from 1966 to February 2010). Results: A 61-year-old female patient with AAV developed cognitive impairment. Cerebrospinal fluid analysis was unremarkable, while magnetic resonance (MR) imaging showed multiple left hemisphere infarctions and MR angiography revealed multiple stenoses of the distal branches of the left median cerebral artery. Treatment with glucocorticoids, cyclophosphamide, and intravenous immunoglobulins led to improvement. CNS vasculitis often arises when vasculitis is active elsewhere. There is no clear preponderance of gender or of age of onset. Both ANCA-positive and-negative cases of CNS vasculitis are documented. The diagnosis is usually based on clinical CNS manifestations and multiple ischaemic (sometimes haemorrhagic) MR lesions mainly affecting the white matter. Angiography is often negative. Treatment with glucocorticoids and cyclophosphamide, sometimes with adjunctive intravenous immunoglobulins, usually improves clinical features and MR lesions. Conclusion: AAV rarely involves the CNS. CNS vasculitis should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if MR shows multiple ischaemic (sometimes haemorrhagic) lesions mainly affecting the white matter. Sepsis, coagulation disorders, and severe hypertension must be ruled out. Awareness of this rare but severe complication can allow early recognition and prompt treatment.

Original languageEnglish
Pages (from-to)759-766
Number of pages8
JournalClinical and Experimental Rheumatology
Volume28
Issue number5
Publication statusPublished - 2010

Fingerprint

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Central Nervous System Vasculitis
Central Nervous System
Magnetic Resonance Spectroscopy
Intravenous Immunoglobulins
Cyclophosphamide
Glucocorticoids
Antineutrophil Cytoplasmic Antibodies
Cerebral Arteries
Magnetic Resonance Angiography
Vasculitis
Neurologic Manifestations
Age of Onset
PubMed
Infarction
Cerebrospinal Fluid
Sepsis
Angiography
Pathologic Constriction
Therapeutics

Keywords

  • Lethargy
  • MR dates
  • Seizures

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

@article{904b790057c34fce9f71d4cb8a64b16f,
title = "Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system: Case report and review of the literature",
abstract = "Objectives: To report a case of biopsyproven, ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and to review the relevant literature. Methods: Descriptive case report of one patient with AAV-related CNS vasculitis and review of the relevant literature (PubMed search from 1966 to February 2010). Results: A 61-year-old female patient with AAV developed cognitive impairment. Cerebrospinal fluid analysis was unremarkable, while magnetic resonance (MR) imaging showed multiple left hemisphere infarctions and MR angiography revealed multiple stenoses of the distal branches of the left median cerebral artery. Treatment with glucocorticoids, cyclophosphamide, and intravenous immunoglobulins led to improvement. CNS vasculitis often arises when vasculitis is active elsewhere. There is no clear preponderance of gender or of age of onset. Both ANCA-positive and-negative cases of CNS vasculitis are documented. The diagnosis is usually based on clinical CNS manifestations and multiple ischaemic (sometimes haemorrhagic) MR lesions mainly affecting the white matter. Angiography is often negative. Treatment with glucocorticoids and cyclophosphamide, sometimes with adjunctive intravenous immunoglobulins, usually improves clinical features and MR lesions. Conclusion: AAV rarely involves the CNS. CNS vasculitis should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if MR shows multiple ischaemic (sometimes haemorrhagic) lesions mainly affecting the white matter. Sepsis, coagulation disorders, and severe hypertension must be ruled out. Awareness of this rare but severe complication can allow early recognition and prompt treatment.",
keywords = "Lethargy, MR dates, Seizures",
author = "A. Ghinoi and G. Zuccoli and N. Pipitone and C. Salvarani",
year = "2010",
language = "English",
volume = "28",
pages = "759--766",
journal = "Clinical and Experimental Rheumatology",
issn = "0392-856X",
publisher = "Clinical and Experimental Rheumatology S.A.S.",
number = "5",

}

TY - JOUR

T1 - Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system

T2 - Case report and review of the literature

AU - Ghinoi, A.

AU - Zuccoli, G.

AU - Pipitone, N.

AU - Salvarani, C.

PY - 2010

Y1 - 2010

N2 - Objectives: To report a case of biopsyproven, ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and to review the relevant literature. Methods: Descriptive case report of one patient with AAV-related CNS vasculitis and review of the relevant literature (PubMed search from 1966 to February 2010). Results: A 61-year-old female patient with AAV developed cognitive impairment. Cerebrospinal fluid analysis was unremarkable, while magnetic resonance (MR) imaging showed multiple left hemisphere infarctions and MR angiography revealed multiple stenoses of the distal branches of the left median cerebral artery. Treatment with glucocorticoids, cyclophosphamide, and intravenous immunoglobulins led to improvement. CNS vasculitis often arises when vasculitis is active elsewhere. There is no clear preponderance of gender or of age of onset. Both ANCA-positive and-negative cases of CNS vasculitis are documented. The diagnosis is usually based on clinical CNS manifestations and multiple ischaemic (sometimes haemorrhagic) MR lesions mainly affecting the white matter. Angiography is often negative. Treatment with glucocorticoids and cyclophosphamide, sometimes with adjunctive intravenous immunoglobulins, usually improves clinical features and MR lesions. Conclusion: AAV rarely involves the CNS. CNS vasculitis should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if MR shows multiple ischaemic (sometimes haemorrhagic) lesions mainly affecting the white matter. Sepsis, coagulation disorders, and severe hypertension must be ruled out. Awareness of this rare but severe complication can allow early recognition and prompt treatment.

AB - Objectives: To report a case of biopsyproven, ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and to review the relevant literature. Methods: Descriptive case report of one patient with AAV-related CNS vasculitis and review of the relevant literature (PubMed search from 1966 to February 2010). Results: A 61-year-old female patient with AAV developed cognitive impairment. Cerebrospinal fluid analysis was unremarkable, while magnetic resonance (MR) imaging showed multiple left hemisphere infarctions and MR angiography revealed multiple stenoses of the distal branches of the left median cerebral artery. Treatment with glucocorticoids, cyclophosphamide, and intravenous immunoglobulins led to improvement. CNS vasculitis often arises when vasculitis is active elsewhere. There is no clear preponderance of gender or of age of onset. Both ANCA-positive and-negative cases of CNS vasculitis are documented. The diagnosis is usually based on clinical CNS manifestations and multiple ischaemic (sometimes haemorrhagic) MR lesions mainly affecting the white matter. Angiography is often negative. Treatment with glucocorticoids and cyclophosphamide, sometimes with adjunctive intravenous immunoglobulins, usually improves clinical features and MR lesions. Conclusion: AAV rarely involves the CNS. CNS vasculitis should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if MR shows multiple ischaemic (sometimes haemorrhagic) lesions mainly affecting the white matter. Sepsis, coagulation disorders, and severe hypertension must be ruled out. Awareness of this rare but severe complication can allow early recognition and prompt treatment.

KW - Lethargy

KW - MR dates

KW - Seizures

UR - http://www.scopus.com/inward/record.url?scp=78650526381&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78650526381&partnerID=8YFLogxK

M3 - Article

VL - 28

SP - 759

EP - 766

JO - Clinical and Experimental Rheumatology

JF - Clinical and Experimental Rheumatology

SN - 0392-856X

IS - 5

ER -