Vascular Involvement in Primary Hyperoxalosis: An Evidence Based Sytematic Overview Over a Fifty Year Span

Background and Aims: Primary hyperoxaluria (PH) is a rare genetic disorder characterized by calcium oxalate nephrocalcinosis leading to renal failure. When renal function is impaired, plasma oxalate concentration increases and extra-renal oxalate deposition occurs. Vascular involvement includes vasculitis and ischemic related morbidity and death. The prevalence of vascular involvement is not known with only a few cases described in the literature. A systematic overview of the literature was performed to identify the demographics, clinical presentation and outcomes of such patients. Methods: A computerized search of MEDLINE (Jan 1950 to May 30, 2007), EMBASE (Jan 1988 to May 2007) and CINAHL (Jan 1982 to May 30, 2007 identified twenty three cases of vascular involvement. Results: The majority of those reported in the literature are young females (74%). Vascular deposition of oxalate crystals presents as skin vasculitis; limb or mesenteric ischemia or gangrene. Stroke is uncommon. The lower limbs are affected more than upper limbs followed by mesenteric vessels. Vasculitic skin manifestations occur in 52% of cases. Death occurred in 8 (38%) of cases (63% abdominal gangrene/sepsis; 37% cardiac). Conclusions: Little information exists on vascular involvement in PH. Morbidity and mortality is high (death and limb loss occurring in 2/3 of patients). Awareness of the vascular presentation is thus important to allow a better understanding. Prospective reporting via a national or international registry on optimal management of vascular involvement in PH is needed.