A case of granulomatous angiitis of the central nervous system associated with amyloid angiopathy

Shintaku, Masayuki; OSAWA, Kotaro; Toki, Junko; Maeda, Ryuei; Nishiyama, T.

doi:10.1007/bf00686094

Cited by 40 publications

(21 citation statements)

References 8 publications

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“…There have been three recent reports of the simulta neous occurrence of amyloid angiopathy and isolated angiitis of the CNS in the same patient; both pathologi cal processes principally affecting the leptomeningeal and cortical vessels [71,72,88], The striking coincidence in the sites affected by each condition, together with the fact that many of the giant cells appeared to contain phagocytosed fragments of amyloid in 1 case, strongly indi cates that the changes are somehow related [88]. It remains unclear whether chronic inflammation leads to the deposition of amyloid, or vice versa, or whether these two pathological processes arise together from a common immunological abnormality.…”

Section: Aetiologymentioning

confidence: 99%

Isolated Angiitis/Angiopathy of the Central Nervous System

Hankey¹

1990

Cerebrovasc Dis

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One hundred cases of isolated angiopathy of the central nervous system (CNS) have been described, the majority of whom (71%) were diagnosed histologically. The pathological findings were those of angiitis, most frequently granulomatous angiitis. These patients should be classified as isolated angiitis of the CNS. The minority of patients (29%) were diagnosed according to clinical and angiographic findings which lack sensitivity and specificity. They represent a heterogeneous group, comprising several cases of probable isolated angiitis of the CNS, some clinical and angiographic syndromes associated with acute hypertension, subarachnoid haemorrhage or migraine and other more obscure ''entities'' such as post-partum cerebral angiopathy and isolated benign cerebral angiopathy. In the absence of a histological diagnosis, it is preferable to classify them as isolated angiopathy of the CNS. Although isolated angiitis of the CNS can be considered a discrete, or at least definable, subset of isolated angiopathy of the CNS, it is unclear whether it is a specific disorder or part of a spectrum of disease or even one of a number of reactions to various antigens. The finding of granulomatous angiitis of the CNS (GACNS) in several patients with various systemic disorders suggests that GACNS may be a non-specific pathological reaction. Until the last decade, the prognosis of isolated angiitis of the CNS was extremely poor. Most patients now survive and return to active lives. The major influence on outcome appears to have been the use of combination corticosteroid and cyclophosphamide therapy, although no randomized controlled trials have been undertaken. Before prolonged treatment with such potent medication is prescribed, it recommended that other causes of CNS vasculitis be carefully excluded and the diagnosis of isolated angiitis of the CNS be established histologically by combined leptomeningeal and parenchymal biopsy. The risks of biopsy would not appear to exceed those of untreated isolated angiitis of the CNS. Multicentre collaboration of interested groups should be encouraged if controlled therapeutic trials are to be achieved.

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Section: Aetiologymentioning

confidence: 99%

Isolated Angiitis/Angiopathy of the Central Nervous System

Hankey¹

1990

Cerebrovasc Dis

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“…71 One intriguing, though rare, association is between CAA and isolated cerebral vasculitis. 72 " 76 The vasculitis has been either similar to that seen with rheumatoid disease 72 (characterized by segmental fibrinoid necrosis, adventitial inflammation, and obliterative endarteritis) or like granulomatous angiitis, with prominent giant cells. 75 " 76 Despite their rarity, these examples are of interest because they suggest a pathogenetic mechanism of CAA in some cases, i.e., chronic inflammation.…”

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confidence: 99%

“…72 " 76 The vasculitis has been either similar to that seen with rheumatoid disease 72 (characterized by segmental fibrinoid necrosis, adventitial inflammation, and obliterative endarteritis) or like granulomatous angiitis, with prominent giant cells. 75 " 76 Despite their rarity, these examples are of interest because they suggest a pathogenetic mechanism of CAA in some cases, i.e., chronic inflammation. An important consideration, however, is that the inflammation (especially the giant cell response) may be in reaction to amyloid, which behaves as a foreign material in the peri vascular region of brain.…”

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confidence: 99%

Cerebral amyloid angiopathy. A critical review.

Vinters¹

1987

Stroke

969

696

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“…To our knowledge, thc first report of a patient present- of leptomeningeal vasculitis with associated amyloid deposition has been reported (16). In another report, cerebral vasculitis and amyloid deposits was described in a patient with longstanding rheumatoid arthritis (17).…”

Section: Discussionmentioning

confidence: 96%

Multiple myeloma-associated amyloidosis and giant cell arteritis

Estrada

Stenzel

Burchette

et al. 1998

Arthritis & Rheumatism

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Primary systemic amyloidosis has been associated with the development of symptoms and clinical features characteristic of polymyalgia rheumatica and/or giant cell arteritis (GCA). Case reports of this clinical entity have been published, stating that the amyloid deposition leads to the symptoms of vasculitis. In this report, we present a second case in the English literature of a patient presenting with multiple myeloma-associated amyloidosis and GCA. This is the first case in which the histopathologic findings are described in enough detail to suggest a pathogenic relationship between the two diseases.

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A case of granulomatous angiitis of the central nervous system associated with amyloid angiopathy

Cited by 40 publications

References 8 publications

Isolated Angiitis/Angiopathy of the Central Nervous System

Isolated Angiitis/Angiopathy of the Central Nervous System

Cerebral amyloid angiopathy. A critical review.

Multiple myeloma-associated amyloidosis and giant cell arteritis

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