Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse
Autor(a) principal: | |
---|---|
Data de Publicação: | 2019 |
Outros Autores: | , , , , , |
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. |
id |
RCAP_352166f48fd345a8b801d33931f8ad74 |
---|---|
oai_identifier_str |
oai:scielo:S0872-01692019000100008 |
network_acronym_str |
RCAP |
network_name_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository_id_str |
7160 |
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 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008 |
url |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100008 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
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 |
repository.mail.fl_str_mv |
|
_version_ |
1799137280155189248 |