L CUTHBERTSON1, A KRELLE1, M JOSE1, S YEW1
1Department of Nephrology, Royal Hobart Hospital, Hobart, Australia
Background: Moyamoya disease is a unique progressive cerebrovascular disease characterised by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. The classic angiographic findings of moyamoya disease have been associated with several other diseases, however, our understanding of these associations is limited.
Case Report: A 29-year-old Vietnamese women with a previous diagnosis of moyamoya syndrome presented with right sided pleuritic chest pain, dyspnea and significant peripheral oedema. CT pulmonary angiogram confirmed the diagnosis of acute pulmonary embolism (PE) within the right middle and lower lobes. Investigations showed a serum albumin of 8g/L, a urine albumin to creatinine (ACR) ratio of 450g/mol and serum creatinine of 76umol/L (eGFR 92mL/min/1.73m2). A diagnosis of acute PE in the setting of nephrotic syndrome was made. The patient was commenced on warfarin, frusemide, perindopril and prednisolone (1mg/kg). Due to anticoagulation therapy for her acute PE, a renal biopsy was not performed. ANA was 1:320 of nucleolar pattern. Anti-PLA2R antibodies, anti-dsDNA and ENA were not detected. C3 and C4 were normal. Renal tract ultrasound was unremarkable with no evidence of renal artery stenosis. The patient responded to steroids with a urine ACR of 110g/mol and serum albumin of 26g/L at 2 months.
Conclusion: We report a case of steroid responsive nephrotic syndrome associated with moyamoya syndrome for which only a handful of case reports exist.
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