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1.
BMJ Case Rep ; 15(6)2022 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-35672051

RESUMO

IgA vasculitis is a rare systemic vasculitis in adults, frequently more severe than in paediatric age. It manifests with cutaneous, articular, gastrointestinal and renal involvement.We present a case of a man in his 40s diagnosed with IgA vasculitis with cutaneous, joint, gastrointestinal and renal disease. Significant proteinuria and renal biopsy findings demonstrating crescentic glomerulonephritis led to the onset of early immunosuppression with corticoid and cyclophosphamide. This case report reflects a case of more severe renal impairment due to IgA vasculitis with good outcome with the chosen therapy. The findings in the renal biopsy after treatment supported the good response to the chosen immunosuppression.


Assuntos
Glomerulonefrite por IGA , Glomerulonefrite Membranoproliferativa , Vasculite por IgA , Adulto , Criança , Ciclofosfamida/uso terapêutico , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite Membranoproliferativa/patologia , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Rim/patologia , Masculino
2.
BMJ Case Rep ; 15(4)2022 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-35396240

RESUMO

Acquired haemophilia A is a rare condition defined by the presence of coagulation inhibitors, which are autoantibodies directed against factor VIII that interfere with its activity. We report a case of a 69-year-old woman that presented with knee haemarthrosis followed by spontaneous retroperitoneal haematoma. On coagulation studies, she presented normal prothrombin time with prolonged activated partial thromboplastin time non-correcting on mixture test, low titers of factor VIII and was detected factor VIII inhibitor that led to diagnosis of acquired haemophilia A. She was managed with supportive measures to control haemorrhage and immunosuppressive therapy to eradicate inhibitors, initially with corticosteroids, with partial transitory response, after which she developed new spontaneous haematomas. Rituximab was started at that time, with a good outcome. The additional aetiological study identified autoimmune thyroiditis and autoimmune pangastritis, an association rarely described in literature.


Assuntos
Doença de Hashimoto , Hemofilia A , Tireoidite Autoimune , Idoso , Autoanticorpos , Fator VIII , Feminino , Doença de Hashimoto/complicações , Hematoma/complicações , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemorragia/etiologia , Humanos , Tireoidite Autoimune/complicações
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