Vasculiti del sistema nervoso

Micrograph of a vasculitic neuropathy
Resin section (plastics) with toluidine blue stain, intermed. magnification

Indice degli argomenti Â» Area medica Â» Reumatologia / Neurologia Â» Vasculiti Â» Vasculiti del sistema nervoso

Indice

Introduzione
Vasculiti del sistema nervoso centrale
- Vasculite primitiva del sistema nervoso centrale
- Vasculiti cerebrali associate a vasculiti sistemiche o altre patologie
Vasculiti del sistema nervoso periferico

Fonti, link esterni, immagini, libri e approfondimenti

H. Almoallim, M. Cheikh (eds.), Skills in Rheumatology, https://doi.org/10.1007/978-981-15-8323-0_20
Al Share, B., Zakaria, A., Hiner, E., Iskenderian, Z., & Warra, N. (2017). Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem. Case reports in neurological medicine, 2017, 3870753. https://doi.org/10.1155/2017/3870753
Arienti F, Franco G, Monfrini E, Santaniello A, Bresolin N, Saetti MC and Di Fonzo A (2020) Microscopic Polyangiitis With Selective Involvement of Central and Peripheral Nervous System: A Case Report. Front. Neurol. 11:269. https://doi.org/10.3389/fneur.2020.00269
Belcour, M., Dontaine, P., Monier, A., Lebrun, L., Salmon, I., De Witte, O., Goldman, S., De TiÃ¨ge, X., & Aeby, A. (2022). Case Report: Interest of Positron Emission Tomography in Pediatric Small Vessel Primary Angiitis of the Central Nervous System. Frontiers in pediatrics, 10, 794294. https://doi.org/10.3389/fped.2022.794294
Berlit P. (2010). Diagnosis and treatment of cerebral vasculitis. Therapeutic advances in neurological disorders, 3(1), 29â€“42. https://doi.org/10.1177/1756285609347123
Campos, A. C., Sarmento, S., Narciso, M., & Fonseca, T. (2023). Primary Central Nervous System Vasculitis: A Rare Cause of Stroke. Cureus, 15(5), e39541. https://doi.org/10.7759/cureus.39541
Lukas, C., Keyvani, K., & BÃ¶rnke, C. (2005). Primary angiitis of the central nervous system presenting with subacute and fatal course of disease: a case report. BMC neurology, 5, 16. https://doi.org/10.1186/1471-2377-5-16
Mazzacane, F., Mazzoleni, V., Scola, E., Mancini, S., Lombardo, I., Busto, G., Rognone, E., Pichiecchio, A., Padovani, A., Morotti, A., & Fainardi, E. (2022). Vessel Wall Magnetic Resonance Imaging in Cerebrovascular Diseases. Diagnostics (Basel, Switzerland), 12(2), 258. https://doi.org/10.3390/diagnostics12020258
Nouh, A., Borys, E., Gierut, A. K., & Biller, J. (2014). Amyloid-Beta related angiitis of the central nervous system: case report and topic review. Frontiers in neurology, 5, 13. https://doi.org/10.3389/fneur.2014.00013
Patzig, M., Forbrig, R., KÃ¼pper, C., Eren, O., Saam, T., Kellert, L., Liebig, T., & SchÃ¶berl, F. (2022). Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings. Journal of neurology, 269(2), 982â€“996. https://doi.org/10.1007/s00415-021-10683-7
Ruland, T., Wolbert, J., Gottschalk, M. G., KÃ¶nig, S., Schulte-Mecklenbeck, A., Minnerup, J., Meuth, S. G., GroÃŸ, C. C., Wiendl, H., & Meyer Zu HÃ¶rste, G. (2018). Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System. Frontiers in neurology, 9, 407. https://doi.org/10.3389/fneur.2018.00407
Smitka, M., Bruck, N., Engellandt, K., Hahn, G., Knoefler, R., & von der Hagen, M. (2020). Clinical Perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS). Frontiers in pediatrics, 8, 281. https://doi.org/10.3389/fped.2020.00281
Strunk, D., Schmidt-Pogoda, A., Beuker, C., Milles, L. S., Korsukewitz, C., Meuth, S. G., & Minnerup, J. (2019). Biomarkers in Vasculitides of the Nervous System. Frontiers in neurology, 10, 591. https://doi.org/10.3389/fneur.2019.00591

Birnbaum, J., & Hellmann, D. B. (2009). Primary angiitis of the central nervous system. Archives of neurology, 66(6), 704â€“709. https://doi.org/10.1001/archneurol.2009.76
Cellucci, T., & Benseler, S. M. (2010). Central nervous system vasculitis in children. Current opinion in rheumatology, 22(5), 590â€“597. https://doi.org/10.1097/BOR.0b013e32833c723d
Collins M. P. (2012). The vasculitic neuropathies: an update. Current opinion in neurology, 25(5), 573â€“585. https://doi.org/10.1097/WCO.0b013e3283580432
de Boysson, H., Zuber, M., Naggara, O., Neau, J. P., Gray, F., Bousser, M. G., Crassard, I., TouzÃ©, E., Couraud, P. O., Kerschen, P., Oppenheim, C., Detante, O., Faivre, A., Gaillard, N., Arquizan, C., Bienvenu, B., NÃ©el, A., Guillevin, L., Pagnoux, C., & French Vasculitis Study Group and the French NeuroVascular Society (2014). Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis & rheumatology (Hoboken, N.J.), 66(5), 1315â€“1326. https://doi.org/10.1002/art.38340
Giannini, C., Salvarani, C., Hunder, G., & Brown, R. D. (2012). Primary central nervous system vasculitis: pathology and mechanisms. Acta neuropathologica, 123(6), 759â€“772. https://doi.org/10.1007/s00401-012-0973-9
Hajj-Ali R. A. (2010). Primary angiitis of the central nervous system: differential diagnosis and treatment. Best practice & research. Clinical rheumatology, 24(3), 413â€“426. https://doi.org/10.1016/j.berh.2009.12.003
Hunder, G. G., Salvarani, C., & Brown, R. D., Jr (2010). Primary central nervous system vasculitis: Is it a single disease?. Annals of neurology, 68(5), 573â€“574. https://doi.org/10.1002/ana.22161
Kraemer, M., & Berlit, P. (2021). Primary central nervous system vasculitis – An update on diagnosis, differential diagnosis and treatment. Journal of the neurological sciences, 424, 117422. https://doi.org/10.1016/j.jns.2021.117422
Rice, C. M., & Scolding, N. J. (2020). The diagnosis of primary central nervous system vasculitis. Practical neurology, 20(2), 109â€“114. https://doi.org/10.1136/practneurol-2018-002002
Rodriguez-Pla, A., & Monach, P. A. (2015). Primary angiitis of the central nervous system in adults and children. Rheumatic diseases clinics of North America, 41(1), 47â€“viii. https://doi.org/10.1016/j.rdc.2014.09.004
Salvarani, C., Brown, R. D., Jr, Calamia, K. T., Christianson, T. J., Huston, J., 3rd, Meschia, J. F., Giannini, C., Miller, D. V., & Hunder, G. G. (2011). Rapidly progressive primary central nervous system vasculitis. Rheumatology (Oxford, England), 50(2), 349â€“358. https://doi.org/10.1093/rheumatology/keq303
Salvarani, C., Brown, R. D., Jr, & Hunder, G. G. (2012). Adult primary central nervous system vasculitis: an update. Current opinion in rheumatology, 24(1), 46â€“52. https://doi.org/10.1097/BOR.0b013e32834d6d76
Suri, V., Kakkar, A., Sharma, M. C., Padma, M. V., Garg, A., & Sarkar, C. (2014). Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature. Folia neuropathologica, 52(2), 187â€“196. https://doi.org/10.5114/fn.2014.43790

Introduzione

Vasculite primitiva del sistema nervoso centrale

La vasculite Ã¨ un quadro clinicopatologico caratterizzato da infiammazione e danno a carico della parete dei vasi sanguigni. Dal punto di vista etiologico, puÃ² essere primitiva o secondaria.

Per quanto riguarda il sistema nervoso, Ã¨ possibile distinguere vasculiti del sistema nervoso centrale e vasculiti del sistema nervoso periferico.

Vasculiti del sistema nervoso centrale

A: Axial diffusion-weighted imaging (DWI) showed hyperintense lesions in the right basal ganglia (arrowhead). B: Fluid attenuated inversion recovery showed broad white matter lesions and severe subcortical white matter lesions. C: T2*-weighted imaging (T2*WI) showed multiple cerebral microbleeds both in the thalamus and subcortical region (arrowheads), in addition to the pre-existing intracerebral hemorrhage (arrows). D: MRA showed no occlusion or stenosis in the major cerebral arteries. E, F: Axial DWI showed multiple hyperintense lesions in the bilateral small vessel territory (arrowheads). G, H: Compared with initial T2*WI (G), cerebral microbleeds became more pronounced in the follow-up MRI (H) (arrowheads). L, left; MRA, magnetic resonance angiography.

Copyright Â© 2022 Hoshi et al. â€œProgressive Small Vessel Disease Burden as a Diagnostic of Central Nervous System-Restricted Microscopic Polyangiitis: A Case Report and Review of the Literature.â€ Cureus (CC BY)

Le vasculiti del sistema nervoso centrale possono essere primitive (vasculite primitiva del sistema nervoso centrale) o associate ad altre condizioni, tra cui vasculiti sistemiche, malattie autoimmuni, neoplasie, infezioni, farmaci, etc.

La diagnosi Ã¨ spesso difficile, basata soprattutto sul quadro clinico, sullâ€™esame del liquor e sullâ€™imaging. La conferma diagnostica richiederebbe biopsia cerebrale o leptomeningea, invasiva e spesso non diagnostica (le lesioni sono tipicamente segmentali e possono non essere visualizzate); per questi motivi viene raramente eseguita.

Vasculite primitiva del sistema nervoso centrale

Sinonimi

Angioite primitiva del sistema nervoso centrale
Angioite isolata del sistema nervoso centrale
Angioite granulomatosa del sistema nervoso centrale

Representative imaging findings in Primary Angiitis of the Central Nervous System patients. (Aâ€“C) Images of patients 1â€“3 with black-blood contrast-enhanced T1 weighted sequences, which show contrast enhancement in the vessel walls (red arrow). (D) Angiography image of PACNS patient 4 with longest duration of diseases showing pathognomonic blood vessel irregularities such as stenosis.

La vasculite primitiva del sistema nervoso centrale Ã¨ una vasculite delle arterie di piccolo e medio calibro limitata al sistema nervoso centrale. Â

Ãˆ rara (incidenza di 2 casi ogni milione di abitanti/anno) e rappresenta solo lâ€™1% delle vasculiti del sistema nervoso centrale.

Lâ€™esordio puÃ² avvenire a qualsiasi etÃ , con un picco tra la IV e la VI decade. Alcuni studi riportano una maggiore incidenza nel sesso maschile.

Lâ€™etiologia della vasculite primitiva del sistema nervoso centrale Ã¨ sconosciuta.

Le lesioni cerebrali sono comunemente bilaterali e possono interessare anche il tronco encefalico e il cervelletto. Solo nel 5-10% dei casi Ã¨ documentabile una singola lesione simil-tumorale.

(A) Multiple, non-specific, T2 hyperintense lesions in a 63- year old patient with suspected primary angiitis of the central nervous system who presented with headache and cognitive impairment
(B) Granulomatous pattern of primary angiitis of the central nervous system. Transmural inflammation involves a muscular artery of the leptomeninges with prominent mononuclear (upper) and granulomatous (lower) adventitial inflammation as well as intimal injury with focal fibrin thrombus formation (hematoxylin and eosin 20Ã—). Courtesy of Dr Carlo Salvarani.
(C) Inflammatory involvement of a small vessel. Courtesy of Dr Leonard H Calabrese.
(D) Multiple areas of irregular stenosis and ectasia in a 44year-old patient with biopsyproven primary angiitis of the central nervous system. Courtesy of Dr Leonard H Calabrese.

Quadro clinico

Lâ€™esordio Ã¨ spesso subacuto o insidioso, con:

Cefalea persistente e ingravescente (65% dei casi)
Encefalopatia (50% dei casi) con deficit cognitivi, sonnolenza, alterazioni dello stato di coscienza

PiÃ¹ raramente lâ€™esordio Ã¨ improvviso, con:

TIA
Ictus ischemico
Crisi epilettiche

Progressivamente possono comparire:

Emiparesi (40%)
Afasia (30%)
Atassia (20%)
Disartria (15%)
Disturbi visivi (10-20%)

Manifestazioni rare comprendono:

Emorragia intracranica
Amnesia
Parkinsonismo
Paralisi dei nervi cranici
Paraparesi o tetraparesi

Talvolta possono essere presenti febbre, calo ponderale e sudorazione notturna.

Diagnosi

Brain magnetic resonance (left)
(A) T2-weighted FLAIR imaging demonstrates bilateral, diffuse hyperintense lesions in the white matter with sparing of the cortical U fibers
Pathology of the brain biopsy (right)
(A)â€‰Hematoxylin and eosin staining shows the development of a non-caseating granuloma with giant cells (arrow). (B)â€‰Staining with anti-CD68 antibodies demonstrates the presence of macrophages within a developing granuloma with giant cells (arrow)

Copyright Â© 2014 Ganta et al. â€œRadial contrast enhancement on brain magnetic resonance imaging diagnostic of primary angiitis of the central nervous system: a case report and review of the literature.â€ Journal of medical case reports, Licensee BioMed Central Ltd. (CC BY 2.0 )

La diagnosi di vasculite primitiva del sistema nervoso centrale Ã¨ di esclusione. La conferma diagnostica richiede biopsia cerebrale o leptomeningea (raramente eseguita).

Rientrano in diagnosi differenziale soprattutto:

Sindrome da vasocostrizione cerebrale reversibile
Aterosclerosi
Angiopatia cerebrale amiloide
Vasculiti sistemiche
Granulomatosi linfomatoide
Linfoma cerebrale primitivo
Sindrome da anticorpi antifosfolipidi
Forme infettive:
- Sifilide
- Malattia di Lyme
- Infezioni da rickettsie
- Altre infezioni batteriche
- HIV
- VZV
- Infezioni fungine
Sarcoidosi
Abuso di cocaina
etc.

Gli esami ematochimici sono nella norma.
La sierologia Ã¨ fondamentale per escludere altre cause (HBV, HCV, malattia di Lyme, sifilide, HIV, ANCA, FR, anti-Sm, etc.). Â

Il liquor presenta anomalie aspecifiche:

Modesta iperprotidorrachia
Modesta pleiocitosi linfocitaria
Glicorrachia normale

Imaging:

Risonanza magnetica: infarti corticali-subcorticali multipli bilaterali (raramente una lesione singola simil-tumorale) e lesioni iperintense della sostanza bianca in T2 e FLAIR; possono essere presenti anche enhancement meningeo e lesioni emorragiche intraparenchimali e/o subaranoidee
Risonanza magnetica della parete dei vasi intracranici (vessel wall magnetic resonance imaging, VW-MRI): consente la diagnosi differenziale tra vasculite e altre condizioni (es. aterosclerosi, sindrome da vasocostrizione cerebrale reversibile, malattia di moyamoya, etc.)
Angio-RM (meno sensibile dellâ€™angiografia): stenosi multifocali e/o aneurismi multipli
Angiografia (invasiva ma sensibile, considerata il gold standard per quanto riguarda lâ€™imaging): stenosi multisegmentarie, occlusioni, aneurismi

Primary angiitis of the central nervous system, in 17-year-old female with progressive unexplained neurological deficits. Axial FLAIR image (A) shows an extensive area of abnormal signal in the right frontoparietal white matter and left peritrigonal white matter, without restricted diffusion (B). SWI (C) depicts foci of magnetic susceptibility in the right caudate and subcortical white matter, compatible with microhemorrhages. After administration of gadolinium (D), multiple bilateral parenchymal nodular foci of enhancement are evident in the areas of abnormal FLAIR signal, with perivascular distribution (arrows). MRA images (E and F) show irregularities in multiple distal arterial branches (arrows), consistent with vasculitis.

Brain magnetic resonance imaging (FLAIR) reveals multiple hyperintense cortico-subcortical lesions, frontal, parietal, and in the right occipital temporal transition.

Brain magnetic resonance angiography showing multiple areas of stenosis most pronounced in the intracranial arterial system, specifically in the M1 and M2 segment of the right middle cerebral artery (arrow), as well as stenosis at superior and anteroinferior cerebellar arteries.

Primary angiitis of the central nervous system
Vessel wall contrast enhancement of the right distal M1 segment; correlating TOF-MRA findings showing high-grade stenosis of the affected segment

Primary angiitis of the central nervous system
(A, C) Marked vessel wall contrast enhancement of the posterior circulation, including the basilar artery (arrow) and left posterior communicating artery (arrowhead). Correlating TOF-MRA findings showing high-grade stenosis of the left posterior communicating artery (E).

Primary angiitis of the central nervous system
TOF-MRA of a patient with primary angiitis of the central nervous system and recurrent ischemic strokes shows stenosis of M1 segment of middle cerebral artery (panel (A), white arrow) with corresponding vivid concentric enhancement of arterial walls in post contrast in T1W black blood sequence (panel (B), white arrows) (* contralateral normal M1 segment).

Allâ€™istologia, le lesioni sono tipicamente segmentali (per questo motivo, il 25-50% delle biopsie risulta non diagnostico). I vasi piÃ¹ colpiti sono quelli di piccolo calibro. Lâ€™aspetto istopatologico Ã¨ variabile:

Vasculite granulomatosa con cellule epitelioidi e cellule giganti multinucleate (la forma piÃ¹ frequente)
Vasculite necrotizzante
Vasculite linfocitaria

Sono molto frequenti anche i depositi di amiloide.

Vasculite primitiva del sistema nervoso centrale, istopatologia

(A, B) Transmural granulomatous inflammation of leptomeningeal arteries composed predominantly of lymphocytes and scattered histiocytes (brackets, H&E stain). (C) Vessel wall with membranous staining (immunohistochemistry staining). (D) Cerebral parenchyma showing a focal vessel with concentric perivascular inflammation consisting of chronic inflammatory cells (arrow, H&E stain).

Leptomeningeal arteries show severe transmural inflammation with lumen obliteration (asterisk). The arrow indicates a fibrinoid necrosis in the vessel wall.

Copyright Â© 2005 Lukas et al. â€œPrimary angiitis of the central nervous system presenting with subacute and fatal course of disease: a case report.â€ BMC neurology, Licensee BioMed Central Ltd. (CC BY 2.0)

Histopathology of the cerebral cortex containing medium sized blood vessel. (a) H&E stain Ã—40 reveals acute inflammation concentrated in vessel wall. (b) H&E stain Ã—400 reveals acute necrotizing vasculitis; dense neutrophilic inflammatory exudate; and necrosis in vessel wall.

Primary Angiitis of the Central Nervous System
Hematoxylin and eosin staining magnified 40Ã— (A) and 200Ã— (B) showing dense perivascular lymphohistiocytic infiltration.

Terapia e prognosi

In assenza di terapia, la prognosi della vasculite primitiva del sistema nervoso centrale Ã¨ infausta.

Il trattamento si basa su:

Corticosteroidi
Immunosoppressori (soprattutto ciclofosfamide)

Sono possibili reliquati neurologici permanenti.

Il 25% circa dei pazienti va incontro a recidiva.

Sono fattori prognostici negativi:

Deficit neurologici focali
Disturbi cognitivi
Infarti cerebrali
Coinvolgimento di vasi di grosso calibro

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Vasculiti cerebrali associate a vasculiti sistemiche o altre patologie

Vasculiti sistemiche primarie:
- Malattia di BehÃ§et
- Vasculiti ANCA-associate
- Arterite a cellule giganti e arterite di Takayasu (raramente)
- Altre vasculiti (raramente)
Vasculiti infettive, soprattutto:
- Batteriche:
  - Infezioni da rickettsie
  - Tubercolosi
  - Sifilide
  - Malattia di Lyme
  - Altre infezioni batteriche
- Virali:
  - VZV
  - HIV
  - CMV
- Fungine:
  - Mucormicosi o ficomicosi
  - Aspergillosi
  - Criptococcosi cerebrale
- Parassitarie:
  - Neurocisticercosi
  - Toxoplasmosi
  - Schistosoma (raramente)
Vasculite lupica (raramente)
Altre vasculiti secondarie
Malattie infiammatorie croniche intestinali (molto raramente)
Abuso di cocaina

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Vasculiti del sistema nervoso periferico

High magnification micrograph of a vasculitic neuropathy. Resin section (plastics) with toluidine blue stain.

La vasculite del sistema nervoso periferico puÃ² essere:

Isolata (20% dei casi)
Associata a vasculiti sistemiche o altre condizioni:
- Poliarterite nodosa
- Vasculiti ANCA-associate, soprattutto granulomatosi eosinofila con poliangioite e poliangioite microscopica
- Vasculite lupica
- Vasculite reumatoide
- Vasculite da farmaci (molto raramente, nelle forme piÃ¹ severe)
- Altre vasculiti secondarie (raramente)

La manifestazione piÃ¹ frequente Ã¨ la mononeurite multipla, dovuta a vasculite dei vasa nervorum, ad esordio spesso improvviso; il coinvolgimento puÃ² estendersi fino ad assumere le caratteristiche di una polineuropatia distale simmetrica.

La forma isolata presenta prognosi migliore rispetto alle forme sistemiche e risponde bene ai corticosteroidi (con o senza ciclofosfamide).

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Reumatologia

Vasculiti secondarie

Indice

Fonti, link esterni, immagini, libri e approfondimenti

Introduzione

Vasculiti del sistema nervoso centrale

Vasculite primitiva del sistema nervoso centrale

Quadro clinico

Diagnosi

Terapia e prognosi

Vasculiti cerebrali associate a vasculiti sistemiche o altre patologie

Vasculiti del sistema nervoso periferico

Condividi:

Correlati

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Miastenia gravis

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