Vasculopatia cerebral na síndrome do anticorpo antifosfolípide primária: relato de dois casos

Siqueira Neto, José Ibiapina; Santos, Antonio C.; Fábio, Soraia R. Cabette; Sakamoto, Américo C.

doi:10.1590/S0004-282X1996000400017

Resumos

Os anticorpos antifosfolípides (aFLs) têm sido associados a várias síndromes neurológicas, destacando-se a grande incidência os infartos celebrais. Os mecanismos responsáveis pelo comprometimento vascular encefálico em pacientes com títulos elevados de aFLs permanecem em discussão. A existência de vasculopatia relacionada a este grupo heterogêneo de imunoglobulinas é, no entanto, inquetionável. Relatamos dois casos de síndrome do anticorpo antifosfolípide primário que apresentaram enfartos cerebrais. Os achados angiográficos no primeiro sugeriam vasculite intracraniana, embora houvesse também estenose proximal sugestiva de trombose "in situ". No segundo paciente a angiografia mostrava obstrução de carótida interna, sem sinais de aterosclerose, e na necrópsia foi confirmada a hipótese de vasculopatia trombótica em múltiplos sítios. Nossos achados servem de modelo para discutirmos as controvérsias existentes a respeito da patogênese deste tipo de vasculopatia. Ou seja, se a lesão primária seria vasculite, trombose ou ambas.

vasculopatia cerebral; infarto cerebral; anticorpos antifosfolípedes

The antiphospholipid antibodies are associated with a large number of neurologic syndromes, cerebral infarct (CI) being the most common of them. In these cases the pathogenesis of the CI is poorly understood and remains controversial; however, the existence of a vasculopathy is indubitable. We report the cases of two young patients with CI and diagnosis of primary antiphospholipid syndrome who were submitted to cerebral angiograms, and one of them to necropsy. In one case the angiographic findings were similar to those of vasculitis in intracranial vessels. In the other case we observed obstruction in internal carotid artery at the angiography that looked like thrombosis in situ; at necropsy we found non-atherosclerotic obstruction in coronary arteries. In summary, is the primary lesion vasculitis, thrombosis, or both? These cases ilustrate the discussion and demonstrate that vasculitic mechanisms may be involved in the vasculopathy of primary antiphospholipid syndrome eventhough trombosis occur more frequently.

cerebral vasculopathy; cerebral infarct; antiphospholipid antibodies

Vasculopatia cerebral na síndrome do anticorpo antifosfolípide primária: relato de dois casos

Cerebral vasculopathy in the primary antiphospholipid syndrome: report of two cases

José Ibiapina Siqueira Neto^I; Antonio C. Santos^II; Soraia R. Cabette Fábio^III; Américo C. Sakamoto^IV

^IM.D., Professor Adjunto Doutor do Departamento de Medicina Clínica da Universidade Federal do Ceará

^IIM.D., Professor Assistente de Neurorradiologia do Departamento de Clínica Médica da Faculdade de Medicina de Ribeirão Preto (FMRP) da Universidade de São Paulo (USP)

^IIIM.D., Médica Assistente do Departamento de Neurologia, Psiquiatria e Psicologia Médica da FMRP/USP

^IVM.D., Professor Associado do Departamento de Neurologia, Psiquiatria e Psicologia Médica da FMRP/USP

RESUMO

Os anticorpos antifosfolípides (aFLs) têm sido associados a várias síndromes neurológicas, destacando-se a grande incidência os infartos celebrais. Os mecanismos responsáveis pelo comprometimento vascular encefálico em pacientes com títulos elevados de aFLs permanecem em discussão. A existência de vasculopatia relacionada a este grupo heterogêneo de imunoglobulinas é, no entanto, inquetionável. Relatamos dois casos de síndrome do anticorpo antifosfolípide primário que apresentaram enfartos cerebrais. Os achados angiográficos no primeiro sugeriam vasculite intracraniana, embora houvesse também estenose proximal sugestiva de trombose "in situ". No segundo paciente a angiografia mostrava obstrução de carótida interna, sem sinais de aterosclerose, e na necrópsia foi confirmada a hipótese de vasculopatia trombótica em múltiplos sítios. Nossos achados servem de modelo para discutirmos as controvérsias existentes a respeito da patogênese deste tipo de vasculopatia. Ou seja, se a lesão primária seria vasculite, trombose ou ambas.

Palavras-chave: vasculopatia cerebral, infarto cerebral, anticorpos antifosfolípedes.

ABSTRACT

The antiphospholipid antibodies are associated with a large number of neurologic syndromes, cerebral infarct (CI) being the most common of them. In these cases the pathogenesis of the CI is poorly understood and remains controversial; however, the existence of a vasculopathy is indubitable. We report the cases of two young patients with CI and diagnosis of primary antiphospholipid syndrome who were submitted to cerebral angiograms, and one of them to necropsy. In one case the angiographic findings were similar to those of vasculitis in intracranial vessels. In the other case we observed obstruction in internal carotid artery at the angiography that looked like thrombosis in situ; at necropsy we found non-atherosclerotic obstruction in coronary arteries. In summary, is the primary lesion vasculitis, thrombosis, or both? These cases ilustrate the discussion and demonstrate that vasculitic mechanisms may be involved in the vasculopathy of primary antiphospholipid syndrome eventhough trombosis occur more frequently.

Key words: cerebral vasculopathy, cerebral infarct, antiphospholipid antibodies.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 14-maio-1996.

Dr. José Ibiapina Siqueira Neto - Av. Historiador Raimundo Girão 444/701 - 60165-050 Fortaleza CE - Brasil.

1. Alarcón-Segovia D, Cardiel MH, Reyes E. Antiphospholipid arterial vasculopathy. J Rheumatol 1989;16:762-767.
2. Alarcón-Segovia D, Sanchez-Guerrero J. Primary antiphospholipid syndrome. J Rheumatol 1989; 16:482-488.
3. Antiphospholipid Antibodies in Stroke Study Group (APASS). Clinical and laboratory findings in patients with antiphospholipid antibodies and cerebral ischemia. Stroke 1990;21:1268-1273.
4. Antiphophohpid Antibodies in Stroke Study Group (APASS). Clinical, radiological, and pathological aspects of cerebrovascular disease associated with antiphospholipid antibodies. Stroke 1993;24:120-123.
5. Bacharach JM, Stanson AW, Lie JT, Nichols DA. Imaging spectrum of thrombo-occlusive disease associated with antiphospholipid antibodies. RadioGraphics 1993;13:417-423.
6. Brey RL, Gharavi AE, Lockshin MD. Neurologic complications of antiphospholipid antibodies. Rheum Dis Clin North Am 1993;19:833-850.
7. Brey RL, Hart RG, Sherman DG, Tegeler CH. Antiphospholipid antibodies and cerebral ischemia in young people. Neurology 1990;40:1190-1196.
8. Coull BM, Levine SR, Brey RL. The role of antiphospholipid antibodies in stroke. Neurol Clin 1992; 10:125-143.
9. DeWitt LD, Caplan LR. Antiphospholipid antibodies and stroke. ANJR 1991;12:454-456.
10. Hughes GRV, Harris NN, Gharavi AE. The anticardiolipin syndrome. J Rheumathol 1986;13:486-489.
11. Hughson MD, McCarty GA, Sholer CM, Bromback RA. Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome. Mod Pathol 1993;6:644-653.
12. Levine SR, Deegan MJ, Futrell N, Welch KMA. Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. Neurology 1990;40:1181-1189.
13. Lie JT. Vasculopathy in the antiphospholipid syndrome: thrombosis or vasculitis or both? J Reumathol 1989; 16:713-715.
14. Lie JT. Vasculitis simulators and vasculitis look-alikes. Curr Opin Rheumatol 1992;4:47-55.
15. Lockshin MD. Which patients with antiphospholipid antibody should treated and how? Rheum Dis Clin North Am 1993;19:235-247.

Datas de Publicação

Publicação nesta coleção
12 Nov 2010
Data do Fascículo
Dez 1996

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Alarcón-Segovia D, Cardiel MH, Reyes E. Antiphospholipid arterial vasculopathy. J Rheumatol 1989;16:762-767.

[2] 2. Alarcón-Segovia D, Sanchez-Guerrero J. Primary antiphospholipid syndrome. J Rheumatol 1989; 16:482-488.

[3] 3. Antiphospholipid Antibodies in Stroke Study Group (APASS). Clinical and laboratory findings in patients with antiphospholipid antibodies and cerebral ischemia. Stroke 1990;21:1268-1273.

[4] 4. Antiphophohpid Antibodies in Stroke Study Group (APASS). Clinical, radiological, and pathological aspects of cerebrovascular disease associated with antiphospholipid antibodies. Stroke 1993;24:120-123.

[5] 5. Bacharach JM, Stanson AW, Lie JT, Nichols DA. Imaging spectrum of thrombo-occlusive disease associated with antiphospholipid antibodies. RadioGraphics 1993;13:417-423.

[6] 6. Brey RL, Gharavi AE, Lockshin MD. Neurologic complications of antiphospholipid antibodies. Rheum Dis Clin North Am 1993;19:833-850.

[7] 7. Brey RL, Hart RG, Sherman DG, Tegeler CH. Antiphospholipid antibodies and cerebral ischemia in young people. Neurology 1990;40:1190-1196.

[8] 8. Coull BM, Levine SR, Brey RL. The role of antiphospholipid antibodies in stroke. Neurol Clin 1992; 10:125-143.

[9] 9. DeWitt LD, Caplan LR. Antiphospholipid antibodies and stroke. ANJR 1991;12:454-456.

[10] 10. Hughes GRV, Harris NN, Gharavi AE. The anticardiolipin syndrome. J Rheumathol 1986;13:486-489.

[11] 11. Hughson MD, McCarty GA, Sholer CM, Bromback RA. Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome. Mod Pathol 1993;6:644-653.

[12] 12. Levine SR, Deegan MJ, Futrell N, Welch KMA. Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. Neurology 1990;40:1181-1189.

[13] 13. Lie JT. Vasculopathy in the antiphospholipid syndrome: thrombosis or vasculitis or both? J Reumathol 1989; 16:713-715.

[14] 14. Lie JT. Vasculitis simulators and vasculitis look-alikes. Curr Opin Rheumatol 1992;4:47-55.

[15] 15. Lockshin MD. Which patients with antiphospholipid antibody should treated and how? Rheum Dis Clin North Am 1993;19:235-247.