<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0871-9721</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Imunoalergologia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Imunoalergologia]]></abbrev-journal-title>
<issn>0871-9721</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Alergologia e Imunologia Clínica]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0871-97212023000300227</article-id>
<article-id pub-id-type="doi">10.32932/rpia.2023.08.119</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[The challenge of omalizumab refractory chronic spontaneous urticaria and the relevance of suspecting Schnitzler syndrome without monoclonal gammopathy]]></article-title>
<article-title xml:lang="pt"><![CDATA[Urticária crónica espontânea refratária ao omalizumab - um desafio diagnóstico - síndrome de Schnitzler sem gamapatia monoclonal]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Paulino]]></surname>
<given-names><![CDATA[Marisa]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Costa]]></surname>
<given-names><![CDATA[Célia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Universitário Lisboa Norte Hospital de Santa Maria Serviço de Imunoalergologia]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Faculdade de Medicina de Lisboa Clinica Universitária de Imunoalergologia ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<volume>31</volume>
<numero>3</numero>
<fpage>227</fpage>
<lpage>232</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0871-97212023000300227&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0871-97212023000300227&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0871-97212023000300227&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[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.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO  Introdução:  A síndrome de Schnitzler (SchS) é uma síndrome autoinflamatória caracterizada por presença de urticária e gamapatia monoclonal (MG). Estão descritos casos em que a MG não está presente.  Caso clínico:  Mulher de 43 anos diagnosticada com urticária crónica espontânea há 2 anos, refratária a terapêutica com omalizumab (OMZ) apesar de aumento de dose até 600mg/mês. Após 6 meses de OMZ iniciou astenia, artralgias e febre vespertina, associadas a elevação dos parâmetros inflamatórios e biópsia cutânea com infiltração neutrofílica. Apesar da ausência de MG, o SchS foi considerado o diagnóstico mais provável, após exclusão dos restantes diagnósticos diferenciais. Suspendeu OMZ e iniciou anakinra, com resolução completa do quadro clínico e laboratorial.  Conclusão:  Este caso demonstra a importância do acompanhamento dos doentes com urticária crónica grave em consulta especializada e a necessidade de rever o diagnóstico. Síndromes autoinflamatórias, como a síndrome de Schnitzler, devem ser incluídas no diagnóstico diferencial.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Autoinflammatory syndromes]]></kwd>
<kwd lng="en"><![CDATA[chronic urticaria]]></kwd>
<kwd lng="en"><![CDATA[schnitzler syndrome]]></kwd>
<kwd lng="en"><![CDATA[urticaria]]></kwd>
<kwd lng="pt"><![CDATA[Síndromes autoinflamatórias]]></kwd>
<kwd lng="pt"><![CDATA[Síndrome de Schnitzler]]></kwd>
<kwd lng="pt"><![CDATA[Urticaria]]></kwd>
<kwd lng="pt"><![CDATA[urticária crónica]]></kwd>
</kwd-group>
</article-meta>
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