RESUMO
Polyangiitis overlap syndrome (POS) is a diagnostic term coined by Leavitt and Fauci that characterises patients with overlapping features of more than one vasculitis. Prior case studies of antineutrophil cytoplasmic antibodies (ANCA)-associated POS have only been published in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis alongside proteinase-3/cytoplasmic (C)-ANCA positivity. We present a case of a 60-year-old woman with dyspnoea, hemoptysis, positive perinuclear-ANCA and renal biopsy demonstrating evidence of microscopic polyangiitis. In addition, our patient also had asthma, mononeuritis multiplex, eosinophilia and migratory pulmonary infiltrates, thus fulfilling the criteria for EGPA. This novel case report suggests that POS is not limited to C-ANCA positivity and has variable presentations.
Assuntos
Síndrome de Churg-Strauss/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Poliangiite Microscópica/diagnóstico , Mononeuropatias/fisiopatologia , Insuficiência Renal Crônica/fisiopatologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Azatioprina/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/imunologia , Síndrome de Churg-Strauss/fisiopatologia , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/fisiopatologia , Pessoa de Meia-Idade , Peroxidase/imunologia , Prednisona/uso terapêutico , Insuficiência Renal Crônica/patologia , Tomografia Computadorizada por Raios XRESUMO
Autoimmune liver diseases (AILD) are a group of immunologically induced hepatic disorders that can lead to liver cirrhosis and end-stage liver disease. Extra-hepatic involvement and association with rheumatic diseases (such as Sjögren's syndrome, systemic sclerosis and rheumatoid arthritis) are well known, whereas the coexistence of AILD with small-vessel vasculitis in the same patients have been only occasionally reported. In the present paper we report four such cases and an extensive review of the literature. Clinical features of autoimmune-liver diseases associated with small-vessel vasculitis are discussed, as well as possible common pathogenic pathways including HLA genomics, costimulatory molecules and autoantibodies. In conclusion, knowledge about this association can help physicians in recognising and treating an aggressive disease which could otherwise result in severe and multiple organ damage, compromising the overall prognosis and the indication to liver transplantation.
Assuntos
Síndrome de Churg-Strauss/imunologia , Granulomatose com Poliangiite/imunologia , Hepatite Autoimune/imunologia , Cirrose Hepática Biliar/imunologia , Poliangiite Microscópica/imunologia , Adulto , Idoso , Síndrome de Churg-Strauss/complicações , Feminino , Granulomatose com Poliangiite/complicações , Hepatite Autoimune/complicações , Humanos , Cirrose Hepática Biliar/complicações , Masculino , Poliangiite Microscópica/complicaçõesRESUMO
Se presenta un caso comprobado de síndrome de Churg-Strauss, el tercero que se aplica en el país, con dos características clínicas interesantes: ausencia de asma bronquial y atopia y compromiso predominate a nivel del aparato gastrintestinal. Se hace notar que la ausencia de asma no excluye el diagnóstico.