Central nervous system vasculitis
Updates to Article Attributes
Central nervous system (CNS) vasculitides represent a heterogeneous group of inflammatory diseases affecting the walls of 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 discuss the primaryprimary angiitis of the CNS (PACNS) since the other vasculitides were already discussed in specific articles.
Terminology
CNS vasculitides are classified as 1-2:
- primary: when it is confined to the CNS with no involvement of other systems - referred as PACNS
- secondary: it occurs in the context of a systemic inflammatory or infectious process
Please, note that this classification is different from that one used when discussing systemic vasculitides.
Epidemiology
PACNS remains a rare disorder: an estimated average annual incidence rate of 2.4 cases per one million person. It affects patients of all ages, but peaks at around 50 years of age, being males affected more commonly than females 1.
Secondary causes of CNS vasculitis far exceed in number PACNS 2. Please refer to each specific vasculitis for further details.
Clinical presentation
Clinical features of PACNS are non-specific. The diagnosis is made based on Calabrese’s criteria 4, including:
- presence of an acquired otherwise unexplained neurological or psychiatric deficit
- presence of either classic angiographic or histopathological features of angiitis within the CNS (biopsy remains the standard of reference for its diagnosis 3)
- no evidence of systemic vasculitis or any disorder that could cause or mimic the angiographic or 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 element is unknown 3.
CNS secondary vasculitides:
- affecting large blood vessels
- Takayasu arteritis - uncommon to have CNS involvement
-
Giantgiant cell arteritis
- affecting medium blood vessels
- affecting small blood vessels
- IgA arteritis
- microscopic polyangiitis (microscopic polyarteritis)
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
- variable vessels sizes
- associated with systemic disease
- SLE
-
Rheumatoidrheumatoid arthritis - Sjogren syndrome
- APLA syndrome
-
Sclerodermascleroderma
- associated with a known aetiology
-
infecctioninfection-induced vasculitis - drug-induced
- malignant-induced
- radiation-induced
-
Radiographic features
Imaging findings for PACNS are usually variable and nonspecific, being the ischemic infarctions the most common lesions, occurring in 53% of cases 5.
CT
May show areas of hypoattenuation.
MRI
Is more specific to show multiple infarctions: usually bilateral, affecting different vascular territories of variable size, and in various stages of healing.
T2 and FLAIR high intensity lesions in the white matter are also very common in PACNS, but completely nonspecific.
Meningeal enhancement and intracranial haemorrhages can also be seen.
Angiography (DSA)
May show 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 by Humberto Cravioto and Irwin Feigin 6.
Differential diagnosis
Practical points
Remember that despite of being composed by nonspecific findings, MRI is almost 100% sensitive for 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 discuss the primary angiitis of the CNS (PACNS) 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>- +<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 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>Takayasu arteritis</li>-<li>Giant cell arteritis </li>- +<li>
- +<a title="Takayasu arteritis" href="/articles/takayasu-arteritis">Takayasu arteritis</a> - uncommon to have CNS involvement</li>
- +<li><a title="Giant cell arteritis (GCA)" href="/articles/giant-cell-arteritis">giant cell arteritis </a></li>
-<li>polyarteritis nodosa (PAN)</li>-<li>Kawasaki disease </li>- +<li>
- +<a title="Polyarteritis nodosa (PAN)" href="/articles/polyarteritis-nodosa-1">polyarteritis nodosa </a>(PAN)</li>
- +<li><a title="Kawasaki disease (KD)" href="/articles/kawasaki-disease">Kawasaki disease </a></li>
-<li>IgA arteritis</li>-<li>microscopic polyangiitis (microscopic polyarteritis)</li>-<li>granulomatosis with polyangiitis</li>-<li>eosinophilic granulomatosis with polyangiitis</li>- +<li><a title="IgA arteritis" href="/articles/iga-arteritis">IgA arteritis</a></li>
- +<li>
- +<a title="Microscopic polyangiitis" href="/articles/microscopic-polyangiitis">microscopic polyangiitis</a> (microscopic polyarteritis)</li>
- +<li><a title="Granulomatosis with polyangiitis" href="/articles/granulomatosis-with-polyangitis">granulomatosis with polyangiitis</a></li>
- +<li><a title="Eosinophilic granulomatosis with polyangiitis (EGPA)" href="/articles/eosinophilic-granulomatosis-with-polyangiitis">eosinophilic granulomatosis with polyangiitis</a></li>
-<li>Cogan syndrome</li>-<li>Behçet disease</li>- +<li><a title="Cogan syndrome" href="/articles/cogan-syndrome">Cogan syndrome</a></li>
- +<li><a title="CNS manifestations of Behçet disease" href="/articles/cns-manifestations-of-behcet-disease-1">Behçet disease</a></li>
-<li>SLE</li>-<li>Rheumatoid arthritis</li>-<li>Sjogren syndrome </li>-<li>APLA syndrome</li>-<li>Scleroderma</li>- +<li><a title="Systemic lupus erythematosus" href="/articles/systemic-lupus-erythematosus">SLE</a></li>
- +<li><a title="Rheumatoid arthritis (RA)" href="/articles/rheumatoid-arthritis">rheumatoid arthritis</a></li>
- +<li><a title="Sjogren Syndrome" href="/articles/sjogren-syndrome-1">Sjogren syndrome </a></li>
- +<li><a title="APLA syndrome" href="/articles/antiphospholipid-syndrome">APLA syndrome</a></li>
- +<li><a title="Scleroderma" href="/articles/scleroderma">scleroderma</a></li>
-<li>infecction-induced vasculitis</li>- +<li>infection-induced vasculitis</li>
References changed:
- 6. Cravioto H, Feigin I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology. 1998;9: 599-609. <a href="http://www.ncbi.nlm.nih.gov/pubmed/13812692">Pubmed citation</a><span class="auto"></span>
- 6. CRAVIOTO H, FEIGIN I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology. 1998;9: 599-609. <a href="http://www.ncbi.nlm.nih.gov/pubmed/13812692">Pubmed citation</a><span class="auto"></span>