RESUMO
The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.
Assuntos
Doença de Hashimoto/complicações , Cirrose Hepática Biliar/complicações , Poliangiite Microscópica/complicações , Síndrome de Sjogren/complicações , Anticorpos Antinucleares/sangue , Biomarcadores/sangue , Biópsia , Feminino , Doença de Hashimoto/sangue , Doença de Hashimoto/diagnóstico , Doença de Hashimoto/tratamento farmacológico , Doença de Hashimoto/imunologia , Humanos , Imunossupressores/uso terapêutico , Cirrose Hepática Biliar/sangue , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/tratamento farmacológico , Cirrose Hepática Biliar/imunologia , Poliangiite Microscópica/sangue , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/imunologia , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Insuficiência Renal/etiologia , Síndrome de Sjogren/sangue , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/imunologia , Esteroides/uso terapêutico , Resultado do TratamentoRESUMO
Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.
