Dr Victoria Sasongko1, Dr Mayy Hamde2, Dr Venkat Manickavasagam1, Dr William James1
1Northern NSW LHD, Lismore, Australia, 2Central Coast LHD, Gosford, Australia
Biography:
Bio to come
Background:
Lupus podocytopathy is an increasingly recognised entity of nephrotic syndrome with histological finding with normal glomeruli with or without mesangial proliferation and diffuse podocyte effacement. The management follows the treatment algorithm for minimal change disease with prednisolone and an additional agent (mycophenolate, azathioprine or calcineurin inhibitor) due to risk of relapse.
Case Report:
A 38-year-old Aboriginal female presented with acute right flank pain with associated subacute ten kilograms weight loss and migratory arthralgias. Her CT-scan revealed right sided renal infarct with associated areas of hypoperfusion in the spleen, liver and thickening of small bowel concerning for multi-organ infarcts.
She has positive speckled ANA > 1:2560, anti-dsDNA 41IU/mL, positive anti-Smith and RNP with low C3. Her Creatinine was 44umol/L, Albumin of 17g/L and urine protein/creatinine of 632mg/mmol consistent with nephrotic syndrome.
Her renal biopsy shows mild segmental increase in mesangial hypercellularity and marked diffuse foot process effacement with full house Immunofluorescence consistent with Lupus Podocytopathy.
Our patient was commenced on rapid taper of Prednisolone due to Cushingoid features. This achieved partial remission with nadir urine albumin/creatinine of 40mg/mmol. Due to concerns of high relapse rate and poor compliance, she was commenced on two doses of Rituximab – 1g two weeks apart. Six months after initial therapy, her CD19 was undetectable, Creatinine was 63umol/L and Albumin of 29g/L although her urine protein/creatinine was elevated at 310mg/mmol. Her repeat renal biopsy showed minimal change disease.
Conclusions:
Lupus podocytopathy is a rare but increasingly recognised cause of nephrotic syndrome in patient with Lupus. The less conventional choice of Rituximab chosen for compliance factor has not been efficacious in preventing relapse.