Central nervous system vasculitis
Updates to Article Attributes
Central nervous system (CNS) vasculitides represent a a heterogeneous group of inflammatory diseases affecting the walls of blood blood vessels in brain, spinal cord, and the meninges.
Please refer to the article on vasculitis for a general discussion of that entity.
The aim of this article will be to discuss theprimary angiitis angiitis of the CNS (PACNS) since the other vasculitides were already discussed in specific articles.
Terminology
CNS vasculitides are are classified as 1-2:
- primary: confined to the CNS with no
involvementinvolvement of other systems - referred as PACNS - secondary: occurs in the context of a systemic inflammatory or infectious
processprocess
Please, note that this classification is different from that one used when discussing systemic vasculitides.
Epidemiology
PACNS remains a rare disorder: an estimated average average annual incidence rate of 2 2.4 cases per million. It affects patients of all ages, but peaks peaks at around 50 years of age, with males affected more commonly than than females 1.
Secondary causes of CNS vasculitis far exceed far exceed in number PACNS 2. Please refer to each specific specific vasculitis for further details.
Clinical presentation
Clinical features of PACNS are non-specific. The diagnosis is made based on Calabrese Calabrese’s criteria 4, including:
- presence
of an acquired otherwise unexplained neurological or psychiatricof an acquired otherwise unexplained neurological or psychiatric deficit - presence of either classic angiographic or histopathological
featuresfeatures of angiitis within the CNS (biopsy remains the standard of reference fordiagnosisdiagnosis 3) - no evidence of systemic
vasculitisvasculitis or any disorder that could cause or mimic the angiographicoror pathological features of the disease
When part of a systemic disorder, the diagnosis may be easier, unless the cerebral symptoms are the first to manifest. Please refer to a specific vasculitis for further details on clinical manifestation.
Pathology
For almost all forms of vasculitis, including PACNS, the triggering factor is unknown 3.
CNS secondary vasculitides:
- affecting large
bloodblood vessels- Takayasu arteritis: uncommon to have CNS involvement
- giant cell arteritis
- affecting medium blood vessels
- affecting small blood vessels
- variable vessels sizes
- associated with systemic disease
- associated with a known aetiology
- infection-induced vasculitis
- drug-induced
- malignant-induced
- radiation-induced
Radiographic features
Imaging findings for PACNS are usually usually variable and nonspecific, with ischemic infarctions the most most common lesions, occurring in 53% of cases 5.
CT
May show areas of hypoattenuation.
MRI
More specific to show multiple infarctions: usually bilateral, affecting different vascular territories of variable variable size, and in various stages of healing.
T2 and FLAIR high intensity lesions in the white matter are also very common in PACNS PACNS, but completely nonspecific.
Meningeal enhancement and intracranial haemorrhage can also be seen.
Angiography (DSA)
Shows focal or multifocal segmental narrowing of both small and medium-sized blood vessels, occlusions are also present. The same findings could be demonstrated in both CTA and MRA.
Treatment and prognosis
PACNS is managed with high dose steroids and cytotoxic agents 3.
History and etymology
PACNS was initially reported in 1959 byHumberto Cravioto Cravioto and Irwin Feigin Feigin 6.
Differential diagnosis
Practical points
Remember that despite of being composed by nonspecific findings, MRI is almost 100 100% sensitive for PACNS PACNS and a normal exam practically excludes this diagnosis 1.
-<p><strong>Central nervous system (CNS) vasculitides </strong>represent a heterogeneous group of inflammatory diseases affecting the walls of blood vessels in brain, spinal cord, and the meninges.</p><p>Please refer to the article on <a href="/articles/vasculitis">vasculitis</a> for a general discussion of that entity. </p><p>The aim of this article will be to discuss the <strong>primary angiitis of the CNS (PACNS) </strong>since the other vasculitides were already discussed in specific articles. </p><h4>Terminology</h4><p>CNS vasculitides are classified as <sup>1-2</sup>:</p><ul>-<li>primary: confined to the CNS with no involvement of other systems - referred as PACNS</li>-<li>secondary: occurs in the context of a systemic inflammatory or infectious process</li>-</ul><p>Please, note that this classification is different from that one used when discussing systemic vasculitides.</p><h4>Epidemiology</h4><p>PACNS remains a rare disorder: an estimated average annual incidence rate of 2.4 cases per million. It affects patients of all ages, but peaks at around 50 years of age, with males affected more commonly than females <sup>1</sup>.</p><p>Secondary causes of CNS vasculitis far exceed in number PACNS <sup>2</sup>. Please refer to each specific vasculitis for further details. </p><h4>Clinical presentation</h4><p>Clinical features of PACNS are non-specific. The diagnosis is made based on Calabrese’s criteria <sup>4</sup>, including:</p><ul>-<li>presence of an acquired otherwise unexplained neurological or psychiatric deficit</li>-<li>presence of either classic angiographic or histopathological features of angiitis within the CNS (biopsy remains the standard of reference for diagnosis <sup>3</sup>)</li>-<li>no evidence of systemic vasculitis or any disorder that could cause or mimic the angiographic or pathological features of the disease</li>- +<p><strong>Central nervous system (CNS) vasculitides </strong>represent a heterogeneous group of inflammatory diseases affecting the walls of blood vessels in brain, spinal cord, and the meninges.</p><p>Please refer to the article on <a href="/articles/vasculitis">vasculitis</a> for a general discussion of that entity. </p><p>The aim of this article will be to discuss the <strong>primary angiitis of the CNS (PACNS) </strong>since the other vasculitides were already discussed in specific articles. </p><h4>Terminology</h4><p>CNS vasculitides are classified as <sup>1-2</sup>:</p><ul>
- +<li>primary: confined to the CNS with no involvement of other systems - referred as PACNS</li>
- +<li>secondary: occurs in the context of a systemic inflammatory or infectious process</li>
- +</ul><p>Please, note that this classification is different from that one used when discussing systemic vasculitides.</p><h4>Epidemiology</h4><p>PACNS remains a rare disorder: an estimated average annual incidence rate of 2.4 cases per million. It affects patients of all ages, but peaks at around 50 years of age, with males affected more commonly than females <sup>1</sup>.</p><p>Secondary causes of CNS vasculitis far exceed in number PACNS <sup>2</sup>. Please refer to each specific vasculitis for further details. </p><h4>Clinical presentation</h4><p>Clinical features of PACNS are non-specific. The diagnosis is made based on Calabrese’s criteria <sup>4</sup>, including:</p><ul>
- +<li>presence of an acquired otherwise unexplained neurological or psychiatric deficit</li>
- +<li>presence of either classic angiographic or histopathological features of angiitis within the CNS (biopsy remains the standard of reference for diagnosis <sup>3</sup>)</li>
- +<li>no evidence of systemic vasculitis or any disorder that could cause or mimic the angiographic or pathological features of the disease</li>
-<li>affecting large blood vessels<ul>- +<li>affecting large blood vessels<ul>
-<li><a href="/articles/giant-cell-arteritis">giant cell arteritis </a></li>- +<li><a href="/articles/giant-cell-arteritis">giant cell arteritis </a></li>
-<li><a href="/articles/kawasaki-disease">Kawasaki disease </a></li>- +<li><a href="/articles/kawasaki-disease">Kawasaki disease </a></li>
-<li><a href="/articles/eosinophilic-granulomatosis-with-polyangiitis">eosinophilic granulomatosis with polyangiitis</a></li>- +<li><a href="/articles/eosinophilic-granulomatosis-with-polyangiitis">eosinophilic granulomatosis with polyangiitis</a></li>
-<li><a href="/articles/sjogren-syndrome-1">Sjogren syndrome </a></li>- +<li><a href="/articles/sjogren-syndrome-1">Sjogren syndrome </a></li>
-<li><a title="infection-induced vasculitis" href="/articles/infection-induced-vasculitis">infection-induced vasculitis</a></li>- +<li><a href="/articles/infection-induced-vasculitis">infection-induced vasculitis</a></li>
-<li>radiation-induced </li>- +<li>radiation-induced </li>
-</ul><h4>Radiographic features</h4><p>Imaging findings for PACNS are usually variable and nonspecific, with <a href="/articles/ischaemic-stroke">ischemic infarctions</a> the most common lesions, occurring in 53% of cases <sup>5</sup>. </p><h6>CT</h6><p>May show areas of hypoattenuation.</p><h6>MRI </h6><p>More specific to show multiple infarctions: usually bilateral, affecting different vascular territories of variable size, and in various stages of healing. </p><p>T2 and FLAIR high intensity lesions in the white matter are also very common in PACNS, but completely nonspecific. </p><p><a href="/articles/leptomeningeal-enhancement">Meningeal enhancement</a> and <a href="/articles/intracranial-haemorrhage">intracranial haemorrhage</a> can also be seen.</p><h6>Angiography (DSA)</h6><p>Shows focal or multifocal segmental narrowing of both small and medium-sized blood vessels, occlusions are also present. The same findings could be demonstrated in both CTA and MRA. </p><h4>Treatment and prognosis</h4><p>PACNS is managed with high dose steroids and cytotoxic agents <sup>3</sup>. </p><h4>History and etymology</h4><p>PACNS was initially reported in 1959 by <strong>Humberto Cravioto</strong> and <strong>Irwin Feigin </strong><sup>6</sup>.</p><h4>Differential diagnosis</h4><ul>-<li><a href="/articles/reversible-cerebral-vasoconstriction-syndrome-2">reversible cerebral vasoconstriction syndromes (RCVS)</a></li>- +</ul><h4>Radiographic features</h4><p>Imaging findings for PACNS are usually variable and nonspecific, with <a href="/articles/ischaemic-stroke">ischemic infarctions</a> the most common lesions, occurring in 53% of cases <sup>5</sup>. </p><h6>CT</h6><p>May show areas of hypoattenuation.</p><h6>MRI </h6><p>More specific to show multiple infarctions: usually bilateral, affecting different vascular territories of variable size, and in various stages of healing. </p><p>T2 and FLAIR high intensity lesions in the white matter are also very common in PACNS, but completely nonspecific. </p><p><a href="/articles/leptomeningeal-enhancement">Meningeal enhancement</a> and <a href="/articles/intracranial-haemorrhage">intracranial haemorrhage</a> can also be seen.</p><h6>Angiography (DSA)</h6><p>Shows focal or multifocal segmental narrowing of both small and medium-sized blood vessels, occlusions are also present. The same findings could be demonstrated in both CTA and MRA. </p><h4>Treatment and prognosis</h4><p>PACNS is managed with high dose steroids and cytotoxic agents <sup>3</sup>. </p><h4>History and etymology</h4><p>PACNS was initially reported in 1959 by <strong>Humberto Cravioto</strong> and <strong>Irwin Feigin </strong><sup>6</sup>.</p><h4>Differential diagnosis</h4><ul>
- +<li><a href="/articles/reversible-cerebral-vasoconstriction-syndrome-2">reversible cerebral vasoconstriction syndromes (RCVS)</a></li>
-</ul><h4>Practical points</h4><p>Remember that despite of being composed by nonspecific findings, MRI is almost 100% sensitive for PACNS and a normal exam practically excludes this diagnosis <sup>1</sup>. </p>- +</ul><h4>Practical points</h4><p>Remember that despite of being composed by nonspecific findings, MRI is almost 100% sensitive for PACNS and a normal exam practically excludes this diagnosis <sup>1</sup>. </p>
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