Malignant renovascular hypertension consequent to IgA vasculitis in a young male patient: a case report.

BACKGROUND

Immunoglobulin A vasculitis (IgAV) is the most common cause of vasculitis among children. However, it is a very rare cause of large artery vasculitis. The histopathology of immunoglobulin A vasculitis typically involves IgA deposits in small vessels of the dermis, joints, kidneys, and gastrointestinal tract. These deposits induce an inflammatory response, activating the complement system, which explains the variety of symptoms and signs.

CASE PRESENTATION: A 14-year-old Levantine Arab male with malignant refractory hypertension and prior left nephrectomy was referred for evaluation. The initial presentation at the age of 4 years revealed severe bilateral renal artery stenosis, left ventricular hypertrophy, and reduced ejection fraction (31%), which improved with medical therapy. Parents declined vascular interventions. At the age of 7 years, left kidney atrophy and total occlusion of the left renal artery led to nephrectomy. At the age of 14 years, hypertension worsened despite multiple antihypertensives. Imaging showed total occlusion of the right renal artery. A multidisciplinary team recommended autotransplantation of the solitary right kidney to the left iliac fossa. Postoperative recovery included transient anuria, followed by normalization of renal function and blood pressure. Histopathology revealed IgA-mediated vascular changes without amyloid deposition. The 1-month follow-up showed controlled blood pressure (130/80 mmHg) on reduced antihypertensive therapy.

CONCLUSION

This rare case sheds light on malignant renovascular hypertension in a young male, possibly due to IgA deposition in the right renal artery after a previous left nephrectomy. The surgical decision took into consideration the young age of the patient and the eventual characteristics of the renal artery, in addition to the size of the artery; therefore, we preferred and proceeded to an auto kidney transplant rather than aortorenal bypass, believing that the arterial anastomosis, which is the most important, could be more convenient from the hemodynamic point of view.