Wünderlich syndrome from lupus-associated vasculitis.

Spontaneous perinephric hemorrhage is a clinically rare life-threatening condition, also known as Wünderlich syndrome. Presentations can be variable, but patients typically display symptoms and signs including flank or abdominal pain, abdominal mass, and hypovolemia. It is important to diagnose the syndrome early because untreated cases carry a high mortality risk, and prompt intervention with an endovascular procedure or surgery is life saving. Causes range from anatomical anomalies, including vascular diseases (vasculitides and aneurysms) and renal tumors, to functional coagulation defect (bleeding tendency). The most common causes of Wünderlich syndrome are renal angiomyolipoma and renal cell carcinoma, constituting 60%~70% of cases. Vascular causes of Wünderlich syndrome are infrequent, and the culprit most frequently is vasculitis resulting from polyarteritis nodosa. Other vasculitides presenting as Wünderlich syndrome are infrequent. We describe a 39-year-old woman with end-stage renal disease from lupus nephritis and spontaneous renal hemorrhage, ascribed to lupus-related vasculitis after serologic testing, computed tomography, and angiographic studies.

Hemojuvelin Predicts Acute Kidney Injury and Poor Outcomes Following Cardiac Surgery.

Acute kidney injury (AKI) is detrimental after cardiac surgery. In this multicenter study, the novel biomarker hemojuvelin (HJV) was evaluated for AKI prediction following cardiac surgery. Urinary HJV, neutrophil gelatinase-associated lipocalin (NGAL), and urinary creatinine were measured in 151 patients after surgery. The outcomes of advanced AKI (KDIGO stages 2 and 3) and all causes of in-hospital mortality as the composite outcome were recorded. Areas under the receiver operator characteristic curves (AUC) and a multivariate generalized additive model (GAM) were applied to predict these outcomes of interest. Urinary HJV differentiated patients with/without AKI, advanced AKI or composite outcome after surgery (p < 0.001, by a generalized estimating equation) in this study. At three hours post-surgery, urinary HJV predicted advanced AKI (p < 0.001) and composite outcome (p < 0.001) with corresponding AUC values of 0.768 and 0.828, respectively. The performance of creatinine-adjusted HJV was also superior to NGAL in predicting advanced AKI (AUC = 0.784 and 0.694; p = 0.037) and composite outcome (AUC = 0.842 and 0.676; p = 0.002). The integration of HJV into the Cleveland Clinic score for advanced AKI led to a significant increase in risk stratification (net reclassification improvement [NRI] = 0.598; p < 0.001).