Serum p-ANCA and MPO-ANCA results were unchanged upon repeat testing two weeks later. The patient’s serum creatinine and urinary protein-creatinine ratio continue to improve since the withdrawal of sulphasalazine,
most recently with a creatinine of 84 μmol/L, and her rheumatoid arthritis remains well-controlled. Conclusion: The present case constitutes a rare instance of sulphasalazine-induced ANCA-vasculitis and pauci-immune GN. Although interstitial nephritis high throughput screening is a well-described consequence of sulphasalazine use, the drug should also be considered in the setting of pauci-immune GN. 296 COINCIDENT IGA NEPHROPATHY IN AN AUSTRALIAN PATIENT WITH FABRY’S DISEASE C RAWLINGS1, L FRANCIS2, A MALLETT1,3, G JOHN1, C DENARO4,5 1Department of Renal Medicine, Royal Brisbane and Women’s Hospital, Brisbane, QLD;
2Department of Anatomical Pathology, Royal Brisbane and Women’s Hospital, Brisbane, QLD; 3CKD.QLD and School of Medicine, University of Queensland, Brisbane, QLD; 4Department of Internal Medicine, Royal Brisbane and Women’s Hospital, Brisbane, QLD; 5Department of Internal Medicine, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia Background: The coincident occurrence of IgA Nephropathy (IgAN) in patients with Fabry’s Disease is being increasingly reported. Understanding of this clinical phenomenon is lacking. This is the first Australian report of such occurrence. Case Report: A 38 year old man with Fabry’s Disease on Enzyme Replacement Therapy (ERT) presented with worsening and refractory proteinuria (urine protein 2.0 g/24 h,
urine protein : creatinine learn more 128 g/mol). eGFR was >90 mL/min/1.73 m2 (CKD-EPI and Nuclear Medicine GFR). Fabry’s Disease was diagnosed at age 25 owing to severe hypertrophic cardiomyopathy and has been on ERT since November 2002. This patient was the index case in his family with several others since diagnosed and ERT commenced. Past medical history includes Interleukin-2 receptor dyslipidemia, hypertension, iatrogenic gynaecomastia (secondary to spironolactone) and thalassaemia minor. Examination findings were of obesity, angiokeratomas, ejection systolic murmur and mild pedal oedema. A renal biopsy was performed demonstrating focal segmental glomerulosclerosis and electron microscopy findings consistent with Fabry’s Disease, however IgA Nephropathy (Oxford Classification M0/S1/E0/T0; mesangial electron-dense deposits) was superimposed. Subsequent treatment has been continued ERT and maximised renin-angiotensin-aldosterone system inhibition. After 1year of further follow up post biopsy, renal function remains unchanged and proteinuria has stabilised (urine protein 2.2 g/24 h, urine protein : creatine 140 g/mol). Conclusions: This coexistence of IgAN with Fabry’s disease and concurrent ERT is an incompletely described phenomenon and the pathogenesis is uncertain.