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Abstract(s)
Introdução: A síndrome de Churg-Strauss foi inicialmente denominada angeíte e
granulomatose alérgica, descrevendo a combinação de uma inflamação eosinofílica,
granulomas extravasculares e vasculite necrozante. A síndrome é uma vasculite
associada a asma e eosinofilia. O atingimento respiratório é marcado principalmente
por uma asma tardia, geralmente severa, resistente à corticoterapia, associada a uma
rinite alérgica e por vezes polipose nasal e rinossinusite. As manifestações
vasculíticas são marcadas por um envolvimento multiorgânico. O prognóstico é
geralmente bom, mas o envolvimento cardíaco e do sistema nervoso central estão
associados a um mau prognóstico. A radiografia do tórax revela frequentemente
infiltrados alveolares de limites mal definidos, por vezes migratórios. Os anticorpos
anticitoplasma dos neutrófilos de tipo perinuclear, são fortemente evocadores do
diagnóstico. Clinicamente o diagnóstico é colocado na presença de asma, eosinofilia
superior a 1500/μl e uma vasculite sistémica com o envolvimento de dois órgãos
extrapulmonares. O diagnóstico necessita frequentemente do recurso à biopsia. O
tratamento repousa sobre a corticoterapia, associada nas formas de mau prognóstico a
imunossupressores (ciclofosfamida).
Objectivo: Expôr um caso clínico com síndrome de Churg-Strauss, com a respectiva
revisão do tema baseada principalmente nos artigos científicos publicados sobre a
síndrome nos últimos vinte anos. Artigos originais foram revistos assim como alguns
tratados de medicina interna, pneumologia e doenças vasculares.
Métodos: Recolha de bibliografia através das bases de dados digitais oferecidas pela
Universidade, nomeadamente a PubMed (através do Portal B-on, da Fundação para a
Computação Cientifica Nacional), com os termos “síndrome de Churg-Strauss”, “angeíte
granulomatosa alérgica” e “vasculite associada aos ANCA”. nos idiomas português,
inglês e francês.
Resultados e Discussão: No presente caso clínico, assume relevância a história de
asma brônquica de difícil controle com múltiplas agudizações e com episódios
recorrentes de hemoptises, num paciente com história de pólipos nasais e rinossinusite
e antecedente de AVC hemorrágico. A eosinofilia elevada orientou a investigação
diagnóstica. O diagnóstico diferencial colocou-se com pneumonia eosinofílica, asma de
difícil controlo e síndrome de hipereosinofilia idiopática. O tratamento com
corticoesteróides foi eficaz e a redução gradual permitiu doses de manutenção baixas.
Conclusão: O diagnóstico da síndrome de Churg-Strauss é raro e o diagnóstico deverá
ser equacionado em todos os pacientes com asma brônquica não controlada e
eosinofilia marcada, para providenciar o tratamento adequado precocemente, o que
pode prevenir o dano orgânico permanente.
Introduction: The Churg-Strauss Syndrome was originally called “allergic granulomatosis and angiitis”, describing the combination of eosinophilic inflammation, extravascular granulomas and vasculitis. The syndrome is a vasculitis associated with asthma and eosinophilia. Asthma, generally severe and corticosteroids resistant, associated to allergic rhinitis and sometimes to nasal polyposis and rhinosinusitis, is the most common sign of respiratory injure. The vasculitis can affect many organs. Although these facts, the prognosis could be good, except if heart or central nervous system were damaged. The chest X-ray shows non-fixed pulmonary infiltrates, with undefined limits. The presence of antineutrophil cytoplasmic antibodies suggests the diagnosis. For the clinical features, the Churg-Strauss Syndrome diagnosis is done in the presence of asthma, eosinophilia greater than 1500/µl and a systemic vasculitis. Biopsy is necessary to confirm the diagnosis. The disease responds well to treatment with steroids, although some patients, with a worse prognosis, benefit from the addition of immunosuppressive agents, particularly cyclophosphamide. Goal: Explain a clinical case and make a review of Churg-Strauss Syndrome from the most recent published articles and textbooks of Internal Medicine, Respiratory Medicine and Vascular Diseases. Methods: A bibliographic search was done in Medline database (through the B-on platform), with the key-words “Churg-Strauss Syndrome”, “allergic granulomatosis angiitis” and “ANCA associated vasculitis”, in Portuguese, in English and in French. Results and Discussion: In this clinical case, the severe asthma, treatment refractory, with multiple exacerbations and some episodes of haemoptysis, is the most important sign of the disease. The patient also has nasal polyposis and rhinosinusitis and a previous history of hemorrhagic stroke. The hypereosinophilia was responsible for the further investigations. Eosinophilic pneumonia, severe asthma and idiopathic hypereosinophilia were the diagnosis hypotheses considered. The treatment with steroids was efficient and the gradual dose-decrease allows a small dosemaintenance. Conclusion: The Churg-Strauss Syndrome is rare and the diagnosis should be excluded in all patients with severe asthma and hypereosinophilia. An early diagnosis provides an early treatment, witch can prevent the permanent organs damage.
Introduction: The Churg-Strauss Syndrome was originally called “allergic granulomatosis and angiitis”, describing the combination of eosinophilic inflammation, extravascular granulomas and vasculitis. The syndrome is a vasculitis associated with asthma and eosinophilia. Asthma, generally severe and corticosteroids resistant, associated to allergic rhinitis and sometimes to nasal polyposis and rhinosinusitis, is the most common sign of respiratory injure. The vasculitis can affect many organs. Although these facts, the prognosis could be good, except if heart or central nervous system were damaged. The chest X-ray shows non-fixed pulmonary infiltrates, with undefined limits. The presence of antineutrophil cytoplasmic antibodies suggests the diagnosis. For the clinical features, the Churg-Strauss Syndrome diagnosis is done in the presence of asthma, eosinophilia greater than 1500/µl and a systemic vasculitis. Biopsy is necessary to confirm the diagnosis. The disease responds well to treatment with steroids, although some patients, with a worse prognosis, benefit from the addition of immunosuppressive agents, particularly cyclophosphamide. Goal: Explain a clinical case and make a review of Churg-Strauss Syndrome from the most recent published articles and textbooks of Internal Medicine, Respiratory Medicine and Vascular Diseases. Methods: A bibliographic search was done in Medline database (through the B-on platform), with the key-words “Churg-Strauss Syndrome”, “allergic granulomatosis angiitis” and “ANCA associated vasculitis”, in Portuguese, in English and in French. Results and Discussion: In this clinical case, the severe asthma, treatment refractory, with multiple exacerbations and some episodes of haemoptysis, is the most important sign of the disease. The patient also has nasal polyposis and rhinosinusitis and a previous history of hemorrhagic stroke. The hypereosinophilia was responsible for the further investigations. Eosinophilic pneumonia, severe asthma and idiopathic hypereosinophilia were the diagnosis hypotheses considered. The treatment with steroids was efficient and the gradual dose-decrease allows a small dosemaintenance. Conclusion: The Churg-Strauss Syndrome is rare and the diagnosis should be excluded in all patients with severe asthma and hypereosinophilia. An early diagnosis provides an early treatment, witch can prevent the permanent organs damage.
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Keywords
Síndrome de Churg-Srauss Vasculite Eosinofilia Asma
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Universidade da Beira Interior