Central Nervous System Involvement in ANCA-Associated Vasculitis

Boysson, Hubert de

doi:10.1007/978-3-030-02239-6_13

Cited by 2 publications

(10 citation statements)

References 33 publications

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“…Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a class of small and medium vessel vasculitidies that affect multiple organ systems including the CNS [ 44 •, 45 ]. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [ 44 •].…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

“…AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [ 44 •]. AAV can cause CNS injury either through inflammation of the small and medium sized cerebral vessels or via extravascular granuloma formation [ 44 •, 45 ]. The most common CNS manifestation is hypertrophic pachymeningitis but posterior reversible encephalopathy syndrome (PRES), parenchymal mass lesions, and ischemic and hemorrhagic stroke can also occur [ 44 •, 45 ].…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

“…AAV can cause CNS injury either through inflammation of the small and medium sized cerebral vessels or via extravascular granuloma formation [ 44 •, 45 ]. The most common CNS manifestation is hypertrophic pachymeningitis but posterior reversible encephalopathy syndrome (PRES), parenchymal mass lesions, and ischemic and hemorrhagic stroke can also occur [ 44 •, 45 ]. The ANCA immunoassay is highly specific in the appropriate clinical context but can be negative in up to half of patients with GPA and MPA [ 44 •].…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

“…The ANCA immunoassay is highly specific in the appropriate clinical context but can be negative in up to half of patients with GPA and MPA [ 44 •]. Biopsy of meningeal and parenchymal tissue is the gold standard of diagnosis [ 44 •, 45 ]).…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

“…General guidelines for acute treatment of AAV depend on whether a clinical attack is considered “organ-threatening” [ 44 •]. CNS involvement generally constitutes classification as organ-threatening or severe and initial treatment includes a combination of high dose steroids and cyclophosphamide [ 44 •, 45 ]. Rituximab is equivalent to cyclophosphamide for acute treatment and superior as a maintenance therapy (level II) [ 46 ].…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

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Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

Lapides

McDonald

2020

Curr Treat Options Neurol

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Purpose of Review This review presents the current recommended therapeutic interventions for inflammatory disease in the central nervous system (CNS) secondary to systemic diseases of immune dysregulation. Treatment recommendations for CNS inflammation associated with rheumatologic conditions, immune-related adverse effects from immune checkpoint inhibitors (ICIs), and demyelinating disease from tumor necrosis factor-α (anti-TNFs) are explored. Additional therapeutic options for inflammation related to postviral syndromes and genetic immunodeficiencies are also discussed. Recent Findings In addition to treatment of mild, moderate, and severe CNS rheumatologic disease as guided by the European League Against Rheumatism (EULAR), early consideration of rituximab for severe IgG4-related disease and induction with anti-TNF therapy for severe neurosarcoidosis should be considered. Although often not first line, treatment options for CNS inflammatory diseases based on disease mechanism are emerging, including tocilizumab for Behcet's disease, natalizumab for ICI associated autoimmune encephalitis, and abatacept for treatment of infiltrative disease secondary to CTLA-4 deficiency. Hematopoietic stem cell treatments represent highly efficacious but risky options for autoimmunity related to genetic immunodeficiency. Summary While early high dose steroids remains first line therapy for most CNS inflammatory conditions, a rapidly expanding arsenal of immune targeted therapies offers clinicians tailored disease specific options for treatment.

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Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

See 3 more Smart Citations

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

Lapides

McDonald

2020

Curr Treat Options Neurol

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From Kidney to Brain: An Uncommon Severe Relapse of Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody (MPO-ANCA) Vasculitis

Araújo

Maia

Massano

et al. 2021

Cureus

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases that affect medium and small blood vessels, with uncommon, variable central nervous system (CNS) involvement. It poses diagnosis challenges due to the limited accuracy of conventional imaging and vast differential diagnosis.We describe the case of a 76-year-old man with a previously diagnosed myeloperoxidase (MPO)-positive AAV with exclusive renal involvement. The patient presented to our emergency department (ED) with sudden-onset weakness of the right side of the body, difficulty speaking, fever, and a history of progressive cognitive impairment in the previous three months (loss of memory, time and space disorientation, acalculia). Brain imaging showed multiple acute and subacute ischemic lesions, suggesting CNS vasculitic involvement. The patient was treated with methylprednisolone pulses, followed by rituximab, with motor and cognitive improvement. Timely diagnosis and adequate treatment of AAV as a cause for new-onset neurological symptoms are crucial to improve outcomes. Otherwise, a higher risk of relapse remains, and extensive neurological deficits may become permanent. Evidence regarding the best treatment options in these patients is scarce and case reports provide further data on this topic.

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Central Nervous System Involvement in ANCA-Associated Vasculitis

Cited by 2 publications

References 33 publications

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

From Kidney to Brain: An Uncommon Severe Relapse of Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody (MPO-ANCA) Vasculitis

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