Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse

Detalhes bibliográficos
Autor(a) principal: Rodrigues,Sara Daniela
Data de Publicação: 2019
Outros Autores: Ventura,Ana Maria, Lopes,Daniela, Pereira,Susana, Almeida,Maria Clara, Gomes,Ana Marta, Fernandes,João Carlos
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008
Resumo: Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis commonly involves kidney, lung, upper respiratory tract, skin, gastrointestinal and occasionally peripheral nervous system. Central nervous system is less commonly affected and is generally part of a multi-organ scenario. We present a case of a 21-year-old female with chronic kidney disease secondary to myeloperoxidase ANCA associated vasculitis, diagnosed at age 19. She then presented with pulmonary-renal syndrome with stage 5 CKD. Three months’ cyclophosphamide and prednisone were instituted, followed by azathioprine, but she remained dialysis dependent. After 2 years, while on maintenance treatment, she was admitted to the emergency department with tonic-clonic seizures. She had started oral ciprofloxacin 2 days before for acute gastroenteritis. No illicit drugs were noticed. Blood tests showed an increased myoglobin but were otherwise unremarkable. No anomalies were reported on cerebral computed tomography or cerebrospinal fluid analysis. Magnetic resonance imaging showed diffuse cortico-subcortical lesions with T2 and proton density hypersignal suggesting recent ischemia, and segmental irregularities of various cerebral and vertebral arteries, consistent with small and medium size vessel vasculitis. Intravenous methylprednisolone and oral cyclophosphamide were started. Anti-myeloperoxidase ANCA levels were increased (>200 RU/mL), reinforcing our diagnostic hypothesis of central nervous system (CNS) recurrence of ANCA vasculitis. The patient had a favorable clinical course, with no neurological sequelae. Conclusions: This is a case of ANCA associated vasculitis that presented with kidney and lung but no CNS involvement and recurred with primarily cerebral disease. Favorable outcome was seen with standard immunosuppression. Awareness of this rare but severe complication is critical for timely recognition and prompt treatment.
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spelling Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapseANCAantineutrophil cytoplasmic antibodyCentral Nervous System VasculitisrelapsevasculitisAntineutrophil cytoplasmic antibody (ANCA) associated vasculitis commonly involves kidney, lung, upper respiratory tract, skin, gastrointestinal and occasionally peripheral nervous system. Central nervous system is less commonly affected and is generally part of a multi-organ scenario. We present a case of a 21-year-old female with chronic kidney disease secondary to myeloperoxidase ANCA associated vasculitis, diagnosed at age 19. She then presented with pulmonary-renal syndrome with stage 5 CKD. Three months’ cyclophosphamide and prednisone were instituted, followed by azathioprine, but she remained dialysis dependent. After 2 years, while on maintenance treatment, she was admitted to the emergency department with tonic-clonic seizures. She had started oral ciprofloxacin 2 days before for acute gastroenteritis. No illicit drugs were noticed. Blood tests showed an increased myoglobin but were otherwise unremarkable. No anomalies were reported on cerebral computed tomography or cerebrospinal fluid analysis. Magnetic resonance imaging showed diffuse cortico-subcortical lesions with T2 and proton density hypersignal suggesting recent ischemia, and segmental irregularities of various cerebral and vertebral arteries, consistent with small and medium size vessel vasculitis. Intravenous methylprednisolone and oral cyclophosphamide were started. Anti-myeloperoxidase ANCA levels were increased (>200 RU/mL), reinforcing our diagnostic hypothesis of central nervous system (CNS) recurrence of ANCA vasculitis. The patient had a favorable clinical course, with no neurological sequelae. Conclusions: This is a case of ANCA associated vasculitis that presented with kidney and lung but no CNS involvement and recurred with primarily cerebral disease. Favorable outcome was seen with standard immunosuppression. Awareness of this rare but severe complication is critical for timely recognition and prompt treatment.Sociedade Portuguesa de Nefrologia2019-03-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008Portuguese Journal of Nephrology & Hypertension v.33 n.1 2019reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008Rodrigues,Sara DanielaVentura,Ana MariaLopes,DanielaPereira,SusanaAlmeida,Maria ClaraGomes,Ana MartaFernandes,João Carlosinfo:eu-repo/semantics/openAccess2024-02-06T17:05:01Zoai:scielo:S0872-01692019000100008Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:01.915213Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
title Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
spellingShingle Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
Rodrigues,Sara Daniela
ANCA
antineutrophil cytoplasmic antibody
Central Nervous System Vasculitis
relapse
vasculitis
title_short Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
title_full Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
title_fullStr Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
title_full_unstemmed Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
title_sort Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
author Rodrigues,Sara Daniela
author_facet Rodrigues,Sara Daniela
Ventura,Ana Maria
Lopes,Daniela
Pereira,Susana
Almeida,Maria Clara
Gomes,Ana Marta
Fernandes,João Carlos
author_role author
author2 Ventura,Ana Maria
Lopes,Daniela
Pereira,Susana
Almeida,Maria Clara
Gomes,Ana Marta
Fernandes,João Carlos
author2_role author
author
author
author
author
author
dc.contributor.author.fl_str_mv Rodrigues,Sara Daniela
Ventura,Ana Maria
Lopes,Daniela
Pereira,Susana
Almeida,Maria Clara
Gomes,Ana Marta
Fernandes,João Carlos
dc.subject.por.fl_str_mv ANCA
antineutrophil cytoplasmic antibody
Central Nervous System Vasculitis
relapse
vasculitis
topic ANCA
antineutrophil cytoplasmic antibody
Central Nervous System Vasculitis
relapse
vasculitis
description Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis commonly involves kidney, lung, upper respiratory tract, skin, gastrointestinal and occasionally peripheral nervous system. Central nervous system is less commonly affected and is generally part of a multi-organ scenario. We present a case of a 21-year-old female with chronic kidney disease secondary to myeloperoxidase ANCA associated vasculitis, diagnosed at age 19. She then presented with pulmonary-renal syndrome with stage 5 CKD. Three months’ cyclophosphamide and prednisone were instituted, followed by azathioprine, but she remained dialysis dependent. After 2 years, while on maintenance treatment, she was admitted to the emergency department with tonic-clonic seizures. She had started oral ciprofloxacin 2 days before for acute gastroenteritis. No illicit drugs were noticed. Blood tests showed an increased myoglobin but were otherwise unremarkable. No anomalies were reported on cerebral computed tomography or cerebrospinal fluid analysis. Magnetic resonance imaging showed diffuse cortico-subcortical lesions with T2 and proton density hypersignal suggesting recent ischemia, and segmental irregularities of various cerebral and vertebral arteries, consistent with small and medium size vessel vasculitis. Intravenous methylprednisolone and oral cyclophosphamide were started. Anti-myeloperoxidase ANCA levels were increased (>200 RU/mL), reinforcing our diagnostic hypothesis of central nervous system (CNS) recurrence of ANCA vasculitis. The patient had a favorable clinical course, with no neurological sequelae. Conclusions: This is a case of ANCA associated vasculitis that presented with kidney and lung but no CNS involvement and recurred with primarily cerebral disease. Favorable outcome was seen with standard immunosuppression. Awareness of this rare but severe complication is critical for timely recognition and prompt treatment.
publishDate 2019
dc.date.none.fl_str_mv 2019-03-01
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dc.publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
dc.source.none.fl_str_mv Portuguese Journal of Nephrology & Hypertension v.33 n.1 2019
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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