DE NOVO HYPERTENSIVE SCLERODERMA RENAL CRISIS IN A RENAL TRANSPLANT PATIENT: CASE REPORT AND REVIEW OF THE LITERATURE

Scleroderma renal crisis (SRC) remains an important contributor to mortality in systemic sclerosis (SSc). Kidney transplant is a cornerstone in management of chronic kidney disease, representing a significant demographic of nephrology patients. Understanding transplant immunology and its interaction with systemic disease is critical to improving transplant success and outcomes. We describe a case of de novo hypertensive SRC in a transplanted kidney, which is, to our knowledge, a clinical manifestation of SSc not yet described in the literature.

A 79 year old female kidney transplant recipient (2020; ESRF secondary to diabetic nephropathy) presented to our renal department in Oct 2024 with fatigue, blue discolouration of the fingers, hypertension and a rise in creatinine from 160 to 300micromol/L. She was immunosuppressed with mycophenolate mofetil (MMF) and tacrolimus; other medications included antihypertensives and long-term moderate dose prednisolone. SSc diagnosis dated to 2022, when she presented with early manifestations of disease such as skin changes, Raynaud’s phenomenon, and non-specific interstitial pneumonitis. Her blood tests were positive for anti-RNA Polymerase III unit autoantibodies, leading to diagnosis. Transplant kidney function remained stable through this period. Around the time of renal presentation, transplant ultrasound (USS) was unremarkable, and other causes of chronic decline in transplant kidney function were considered. Accordingly, IV methylprednisolone was started as an inpatient. Biopsy was taken, and the patient was discharged awaiting results.

Biopsy demonstrated an acute arterial thrombotic microangiopathy (TMA), with no evidence of rejection (Figure 1); in the absence of other causes of acute TMA (such as drug toxicity or antibody-mediated rejection), a diagnosis of SRC was made. Kidney function declined further, and the patient re-presented with a stage 3 AKI on CKD. Repeat USS of the transplant kidney revealed globally reduced perfusion with high velocity in the transplant artery with possible stenosis (Figure 2). Management included iloprost and an increased dose of MMF. Prednisolone was stopped and ramipril commenced. Despite this, the patient sadly died in Feb 2025.

We report novel evidence that hypertensive SRC in a transplant kidney can occur independently of native kidney SRC. Anti-RNA Polymerase III autoantibodies are a strong predictor of SRC development; our case suggests this is likely to extend to de novo transplant SRC. Glucocorticoids are implicated as a significant risk factor in the development of SRC, and in exacerbating established disease. Early and accurate diagnosis can reduce mismanagement with glucocorticoid therapy; nephrologists should hold a low diagnostic threshold. Tacrolimus itself can cause endothelial injury and TMA; concomitant use with glucocorticoids may create a predisposing environment for SRC within a transplanted kidney. Optimal treatment strategies remain unclear; however, emerging classifications of SRC that group cases by pathogenesis rather than hypertensive or normotensive presentations may guide more targeted and specific treatments. Applying these groupings to cases of de novo transplant SRC could direct management approaches at the time of diagnosis.