Abstract
Collapsing variant (CV) of focal segmental glomeruoscrelosis (FSGS) is characterized by collapse of the glomerular tuft with marked proliferation of overlying glomerular visceral epithelial cells. Idiopathic FSGS-CV has been known to display black racial predominance, a higher serum creatinine, more severe features of nephrotic syndrome and a rapidly progressive course to renal failure. It has been clinically refractory for various treatment modalities. Thus, the optimal therapy for this variant is unknown.
We report three cases of this variant, without clinical or serological evidence of HIV-1 infection. All patients clinically showed severe hypertension and steroid resistance at the conventional dose, but two of them attained complete remission after methylprednisolone pulse therapy combined with ciclosporin A. At that time, the improvement of severe hypertension that was achieved by the use of enalapril, losartan, continuous intravenous infusion of nicardipine chloride and timely removal of excess fluid using peritoneal dialysis, was necessary to avoid hypertensive encephalopathy and perform safely the therapy.
In Japan, few cases of this type of FSGS have been reported. However, the incidence of CV has progressively increased over the past two decades at a single center in the United States. So, in the near future we might be confronted with many cases of FSGS-CV and need the optimal therapy.
