Nonamyloidotic fibrillar glomerulopathy and recurrent gestational anasarca

Congo-red-negative microfibrils have been described in various glomerular diseases, some of which have no known etiology. We report the unusual case of a young woman who, over a period of 17 years, developed recurrent gestational anasarca but was asymptomatic between pregnancies except for proteinuria. Her blood pressure and renal function have remained normal over the years. A renal biopsy done 5 years after her third pregnancy showed diffuse mesangial expansion and irregular thickening of the glomerular basement membrane, both caused by the deposition of nonamyloidotic microfibrils. We discuss the differential diagnosis of this case and review the pertinent literature.