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Chulalongkorn Medical Journal

Abstract

Amyloidosis is the disease caused by extra-cellular accumulation of amyloid substancewithin various organs leading to progressive dysfunction of the organs. The organs that aremore commonly involved include the kidney and the heart. This report is aimed topresent acase of systemic amyloidosis in a 69-year-old Thai woman who presented with edema andheavy proteinuria. She was referred to our hospital because of anasarca, suspected ofamyloidosis. Her underlying diseases included diabetes mellitus, hypertension, hypothyroidismand chronic kidney disease stage 4 which were regularly and well controlled with medications.Thephysical examination confirmed the anasarca. Her vital signs were unremarkable. The bloodtests revealed Hb10.2 g%, creatinine 1.98 mg%, FBS 96 mg%, HbA1c 5.6 %, albumin 2.4 g%,globulin 3.1 g%, cholesterol 149 mg%, positive ANA, FT4 1.18 mcg/dl, FT3 1.34 ng/ml, TSH3.916 mIU/ml. The urinalysis showed no redblood cell, no white blood cell, no sugar, protein 4+and the calculated urine protein to creatinine ratio (UPCR) was 8.8. The chest film revealeddiffuse cardiomegaly and bilateral pleural effusion. The echocardiography showed granularsparkling at the interventricular septum which was highly specific for amyloidosis of the heartand moderate pericardial effusion. The abdominal fat pad biopsy was performed and foundpositive for Congo red with apparent apple green birefringence under polarized microscope.She was definitely diagnosed as nephrotic syndrome because of the systemic amyloidosisinvolving the heart and the kidney, not due to the diabetes, hypertension or systemic lupuserythematosus. Generally amyloidosis involving the kidney mostly accumulatesthe amyloidsubstance within the glomerulus and less commonly in the interstitium therefore the mainmanifestation is proteinuria which may vary from minimally asymptomatic to heavy proteinuria,20 - 30 gram a day, accompanied by edema. If the patients are left untreated, the disease willprogress to progressive kidney impairment and mortality.

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