WCN23-0177 Acute Kidney Injury in Scleroderma – It's Not Always Renal Crisis

Introduction: We present an interesting case of ANCA positive vasculitis in the context of systemic sclerosis (SS). This case is notable, as ANCA positive vasculitis and scleroderma renal crisis (SRC) are serious complications of SS. They can present similarly but are managed differently. We highlight the importance of considering pauci-immune glomerulonephritis (GN) in the context of SS. A 48-year-old male was referred due to rising creatinine on routine monitoring blood tests in September 2022. He had a background of limited cutaneous SS, scleroderma-associated pulmonary fibrosis and type two diabetes. Medications included mycophenolate mofetil (MMF), co-trimoxazole, losartan, omeprazole, prednisolone, nintedanib and salbutamol. He was treated for SS with rituximab and cyclophosphamide in 2009. On further questioning, he experienced symptoms of a lower respiratory tract infection, including fevers, shortness of breath and cough, two weeks previous to the blood test. On review, however, these symptoms had settled. He also complained of recurrent episodes of epistaxis a few months. His eGFR had dropped to 45 ml/min/1.73m2 from a baseline of 88 ml/min/1.73m2 in June 2022. Creatinine was 156 micromol/l from a baseline of 89 micromol/l. Method(s): We have compiled this case study by using the patient's clinical notes. Result(s): On examination, he was euvolaemic. His blood pressure was 124/83. There were non-blanching erythematous patches on the palmar aspect of his hands bilaterally. (image. 1). An ultrasound scan of his renal tract showed kidney sizes of 11.5cm on the right and 11.1cm on the left. There was no evidence of hydronephrosis. Urine dip was performed showing significant haemoproteinuria (3+blood & 3+ protein). His urinary albumin creatinine ratio was 172.2 mg/mmol, CRP was 48 mg/l, haemolytic anaemia screen was normal and his anti-neutrophil cytoplasmic antibody (ANCA) was positive with a raised myeloperoxidase (MPO) titre of 74.9 U/ml (normal range 0-9 U/ml). GBM antibody was negative. He was commenced on intravenous methylprednisolone and booked for a renal biopsy. Biopsy showed pauci-immune crescentic necrotising GN with no evidence of thrombotic microangiopathy. He was given rituximab 1gm infusions (2 doses day '0' & day '14') and by the end of October, his renal function had improved to 39 ml/min/1.73m2 from a nadir of 31 ml/min/1.73m2. [Formula presented] Conclusion(s): SS is a multisystem disorder and commonly affects the kidney. Renal impairment associated with SS includes nephritis due to the use of nonsteroidal anti-inflammatory drugs, heart failure causing cardiorenal syndrome or dehydration due to alimentary tract involvement of SS. Scleroderma renal crisis (SRC) is a severe syndrome and occurs in 5-20% of patients with diffuse systemic sclerosis. It commonly presents with hypertension, acute kidney injury (AKI) and an unremarkable urine dip. It can progress to end stage renal failure and is treated primarily with angiotensin converting enzyme inhibition. Conversely, there is an association with ANCA positive vasculitis and SS. It can also present with hypertension and AKI. Here we highlight the diagnostic uncertainty of AKI in the context of SS and the importance of investigating with biopsy for vasculitis in selected patients.

Citation

Brown A., Singh A., Moodley P. & Cornish T. (2023). WCN23-0177 Acute Kidney Injury in Scleroderma – It's Not Always Renal Crisis. Kidney International Reports, 8(3 Supplement), S58. https://doi.org/10.1016/j.ekir.2023.02.128

DOI

10.1016/j.ekir.2023.02.128

URI

https://hdl.handle.net/20.500.14709/1136

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CC BY-NC-ND 4.0

License

https://creativecommons.org/licenses/by-nc-nd/4.0/