NONAMYLOID FIBRILLARY GLOMERULOPATHY - A BRIEF REVIEW OF THE LITERATURE

Nonamyloid fibrillary glomerulopathy is a newly recognized primary glo merulopathy characterized by electron microscopic evidence of either r andom fibrillar deposits (fibrillary glomerulopathy) or organized arra ys of microtubules (immunotactoid glomerulopathy) in the glomerular ca pillary wall and mesangium. The fibrils can be distinguished from amyl oid, sclerosing glomerular disease, and collagen glomerulopathty by th eir size characteristics and lack of reactivity to conventional histoc hemical staining e.g. Congo-red, Thioflavin T. Whether differences in the ultrastructural appearance of these fibrils (fibrillary or microtu bular) portends a different clinical course remains the subject of ong oing debate. The light microscopic features of nonamyloid fibrillary g lomerulopathy are non-diagnostic and include mesangial expansion, mesa ngial hypercellularity, or membranous and membranoproliferative change s. Clinically, patients may present with hypertension (60%), hematuria (75%), nephrotic syndrome (61%), and renal insufficiency (47%). Appro ximately 50% of these patients will progress to end stage renal diseas e over a period of 2 to 4 years. An occasional patient may also presen t with acute crescentic rapidly progressive glomerulonephritis (RPGN). Currently no effective therapy exists for this disease, although, a h andful of patients with nonamyloid fibrillary glomerulopathy complicat ed by acute crescentic RPGN have responded to corticosteroid and/or cy totoxic therapy.