Antiphospholipid syndrome and nephrotic syndrome: are they related?

• Autor(a) principal: Lança,Alice
• Data de Publicação: 2015
• Outros Autores: Escoli,Rachele, Ferrer,Francisco, Andrade,Sequeira
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692015000300011
• topic: Antiphospholipid syndrome; minimal change disease; nephrotic syndrome

The antiphospholipid syndrome is a clinical entity that presents as arterial or venous thrombotic events in association with circulating phospholipid autoantibodies. Patients may display a constellation of neurologic, cardiovascular, obstetric and renal complications as a result of antibody-induced vessel injury. Kidneys are one of the most affected organs and its involvement often reflects the extension of the thrombotic process in its vasculature. However, in addition to the characteristic vascular findings of antiphospholipid nephropathy, an expanding spectrum of renal lesions has been reported. Primary glo- merulopathies may often overcome the clinical picture and present themselves as nephrotic syndrome with or without renal dysfunction. Here, we report a case of a 45-year old woman with antiphospholipid syndrome that presented to the emergency department with generalized oedema. After the initial workup nephrotic syndrome was assumed and a renal biopsy was performed. No detectable glomerular abnormalities other than sclerosis as a result of subcapsular ischaemia were seen. Immunofluorescence was negative for glomeruli deposits. The paucity of lesions in the non-sclerosed glomeruli pointed a primary podocytopathy as the most probable cause. The patient was started on corticosteroids with resultant complete remission in about 8 weeks. Nonetheless, during steroid tapering, the nephrotic syndrome relapsed and a higher dose was again needed for remission. However, the patient had to remain on low-dose corticosteroids for maintenance of nephrotic syndrome regression. Its sudden onset, together with the histological features and the good steroid response, made minimal change disease the most probable diagnosis. Yet, it remains unclear whether minimal change disease was related with antiphospholipid syndrome or was an unfortunate event.