Idiopathic glomerular capillary endotheliosis in a male patient with neophrotic syndrome: response to combination therapy with prednisone and nitrogen mustard

Nephritic syndrome [NS] in patients with glomerular capillary endotheliosis is rare; most of the few reported cases have had preeclampsia. We present an interesting case of idiopathic glomerular capillary endotheliosis in a male patient with NS. A renal biopsy showed capillary endothelial swelling, occlusion of capillary loops, fusion of epithelial cell foot processes, no electron-dense deposits, and no tubulo-interstitial and arteriolar changes. We administered a novel combination therapy with prednisone and nitrogen mustard to combat the endotherlial dysfunction. The systemic symptoms and proteinuria gradually disappeared. This case is the first instance of successful treatment with prednisone plus nitrogen mustard in idiopathic glomerular capillary endotheliosis, and also provides evidence for the superiority of prednisone and nitrogen mustard-based regimens over prednisone alone