Multiple Spontaneous Remitting and Relapsing Nephrotic Syndrome in a Cirrhotic Patient

• Autor(a) principal: Cacheira,Eunice
• Data de Publicação: 2022
• Outros Autores: Laranjinha,Ivo
• 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=S0872-01692022000400237
• topic: Liver Cirrhosis/complications; Nephrosis, Lipoid; Nephrotic Syndrome/complications

ABSTRACT Reports of spontaneous remissions in minimal change disease are rare and often associated with secondary causes. We report the case of a 47-year-old male patient with HIV-HBV/HDV co-infection and cirrhosis, admitted in the emergency department with nephrotic syndrome (hypoalbuminemia 1.2 g/dL and nephrotic range proteinuria 14 440 mg/g of creatinine). Ultrasound showed normal kidneys. Viral load for HIV, HBV, HDV and anti-HCV were negative. Immunologic and dysproteinemia study was normal and there were no signs of malignancy on body computer tomography scan. Abdominal fat pad biopsy was Congo red-stain negative. After initiating an angiotensin-converting enzyme inhibitor, the proteinuria decreased to 3891 mg/g. A kidney biopsy was scheduled, but the patient abandoned follow-up. Two years later, he presented again in anasarca, with mild elevation of transaminases, severe hypoalbuminemia (0.7 g/dL), acute kidney injury (creatinine 2.7 mg/dL) and heavy proteinuria (37 g/24 h). One month after the onset, when referred to a nephrologist, he was in complete remission. A year later, he presented a third episode of abrupt onset nephrotic syndrome. Kidney biopsy revealed minimal change disease. Corticosteroid therapy was withheld, and after one month the patient was again in complete remission. We hypothesize that, in the context of cirrhosis, the clearance of a putative permeability circulating factor may be diminished; hemodynamic changes in renal perfusion in decompensated chronic liver disease may interfere in the glomerular barrier function; and a genetic polymorphism related to podocyte dysfunction could precipitate a nephrotic syndrome that resolves once the liver function resumes its steady state. This case represents an unprecedented report of a minimal change disease in a cirrhotic patient, presenting with recurring episodes of nephrotic syndrome followed by early spontaneous remission.