Normocholesterolemic nephrotic syndrome of systemic lupus erythematosus

Abstract

Although hypercholesterolemia is so often associated with the nephrotic syndrome that it has been included in most definitions of this complex state, when nephrosis is associated with systemic lupus erythematosus the serum cholesterol level may be normal. Among eighteen patients with systemic lupus erythematosus and the nephrotic syndrome, five had the normocholesterolemic variant. Serum cholesterol levels were normal at the height of nephrosis, when serum albumin was at its nadir; in the cases in which complete low density lipoproteins were analyzed, these values also were normal. Patients with systemic lupus erythematosus and normocholesterolemic nephrotic syndrome (mean cholesterol level, 176 mg. per 100 ml.) were compared with patients having systemic lupus erythematosus and hypercholesterolemic nephrotic syndrome (mean cholesterol level, 435 mg. per 100 ml.). Those in the normocholesterolemic group were older, and had a slightly higher incidence of azotemia. The average serum albumin level was identical in the two groups, and the incidence of hypertension was similar. The prognosis in those with normocholesterolemic nephrotic syndrome was more grave than in those with hypercholesterolemic nephrotic syndrome; most patients in the former group died within four months, whereas more than half of the patients with hypercholesterolemic nephrotic syndrome were alive after one year. Renal failure was the major cause of death in both groups. The average serum cholesterol in lupus nephrosis, including the hypercholesterolemic variant, was lower than in the nephrotic syndrome due to other causes. Thus the serum cholesterol level may be of diagnostic value in the occasional patient in whom the nephrotic syndrome is the sole manifestation of systemic lupus erythematosus. Since normocholesterolemic nephrotic syndrome fits all criteria consistent with the nephrotic syndrome, it is suggested that the term “pseudonephrotic syndrome” be dropped and that the variant be referred to as “normocholesterolemic nephrotic syndrome.”