The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy

Detalhes bibliográficos
Autor(a) principal: Paulino,Marisa
Data de Publicação: 2023
Outros Autores: Costa,Célia
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=S0871-97212023000300227
Resumo: ABSTRACT Introduction: Schnitzler syndrome (SchS) is a rare autoinflammatory syndrome characterized by chronic urticaria and monoclonal gammopathy (MG). Clinical cases without monoclonal gammopathy have been recorded. Clinical Case: We report on the case of a 43-year-old female with chronic spontaneous urticaria refractory to omalizumab (OMZ) despite a dosage increase (600 mg/4 weeks). Additionally, she developed asthenia and episodes of arthralgias and fever predominantly in the evening. Laboratory re-evaluation revealed elevated inflammatory parameters and skin biopsy showed neutrophilic infiltration. Although MG was absent, SchS was the most likely diagnosis, as other differential diagnoses were excluded. Treatment was started with anakinra, with complete resolution of symptoms. Conclusion: We highlight the importance of specialized urticaria clinics and of reassessing the diagnosis in patients with refractory chronic urticaria. An autoinflammatory disease, such as SchS, could be the diagnosis, even without the presence of monoclonal gammopathy.
id RCAP_21bd2543de90f361e77f51a46f913035
oai_identifier_str oai:scielo:S0871-97212023000300227
network_acronym_str RCAP
network_name_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository_id_str 7160
spelling The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathyAutoinflammatory syndromeschronic urticariaschnitzler syndromeurticariaABSTRACT Introduction: Schnitzler syndrome (SchS) is a rare autoinflammatory syndrome characterized by chronic urticaria and monoclonal gammopathy (MG). Clinical cases without monoclonal gammopathy have been recorded. Clinical Case: We report on the case of a 43-year-old female with chronic spontaneous urticaria refractory to omalizumab (OMZ) despite a dosage increase (600 mg/4 weeks). Additionally, she developed asthenia and episodes of arthralgias and fever predominantly in the evening. Laboratory re-evaluation revealed elevated inflammatory parameters and skin biopsy showed neutrophilic infiltration. Although MG was absent, SchS was the most likely diagnosis, as other differential diagnoses were excluded. Treatment was started with anakinra, with complete resolution of symptoms. Conclusion: We highlight the importance of specialized urticaria clinics and of reassessing the diagnosis in patients with refractory chronic urticaria. An autoinflammatory disease, such as SchS, could be the diagnosis, even without the presence of monoclonal gammopathy.Sociedade Portuguesa de Alergologia e Imunologia Clínica2023-09-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0871-97212023000300227Revista Portuguesa de Imunoalergologia v.31 n.3 2023reponame: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=S0871-97212023000300227Paulino,MarisaCosta,Céliainfo:eu-repo/semantics/openAccess2024-02-06T17:04:37Zoai:scielo:S0871-97212023000300227Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:18:48.159761Repositório 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 The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
title The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
spellingShingle The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
Paulino,Marisa
Autoinflammatory syndromes
chronic urticaria
schnitzler syndrome
urticaria
title_short The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
title_full The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
title_fullStr The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
title_full_unstemmed The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
title_sort The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy
author Paulino,Marisa
author_facet Paulino,Marisa
Costa,Célia
author_role author
author2 Costa,Célia
author2_role author
dc.contributor.author.fl_str_mv Paulino,Marisa
Costa,Célia
dc.subject.por.fl_str_mv Autoinflammatory syndromes
chronic urticaria
schnitzler syndrome
urticaria
topic Autoinflammatory syndromes
chronic urticaria
schnitzler syndrome
urticaria
description ABSTRACT Introduction: Schnitzler syndrome (SchS) is a rare autoinflammatory syndrome characterized by chronic urticaria and monoclonal gammopathy (MG). Clinical cases without monoclonal gammopathy have been recorded. Clinical Case: We report on the case of a 43-year-old female with chronic spontaneous urticaria refractory to omalizumab (OMZ) despite a dosage increase (600 mg/4 weeks). Additionally, she developed asthenia and episodes of arthralgias and fever predominantly in the evening. Laboratory re-evaluation revealed elevated inflammatory parameters and skin biopsy showed neutrophilic infiltration. Although MG was absent, SchS was the most likely diagnosis, as other differential diagnoses were excluded. Treatment was started with anakinra, with complete resolution of symptoms. Conclusion: We highlight the importance of specialized urticaria clinics and of reassessing the diagnosis in patients with refractory chronic urticaria. An autoinflammatory disease, such as SchS, could be the diagnosis, even without the presence of monoclonal gammopathy.
publishDate 2023
dc.date.none.fl_str_mv 2023-09-01
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=S0871-97212023000300227
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0871-97212023000300227
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0871-97212023000300227
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 Alergologia e Imunologia Clínica
publisher.none.fl_str_mv Sociedade Portuguesa de Alergologia e Imunologia Clínica
dc.source.none.fl_str_mv Revista Portuguesa de Imunoalergologia v.31 n.3 2023
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_ 1799137278292918272

Registros relacionados