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Immunoglobulin Light (Heavy)-Chain Deposition Disease: From Molecular Medicine to Pathophysiology-Driven Therapy

Pierre Ronco^*, Emmanuelle Plaisier^*, Béatrice Mougenot^*, and Pierre Aucouturier

INSERM UMR S ^* 702 and 712; Université Pierre et Marie Curie-Paris 6; Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France

Address correspondence to: Dr. Pierre Ronco, Nephrology and Dialysis Department, Hôpital Tenon, 4 rue de la Chine, 75020 Paris, France. Phone: +33-1-5601-6639; Fax: +33-1-5601-6999; E-mail: pierre.ronco{at}tnn.aphp.fr

Light-, light- and heavy-, and heavy-chain deposition diseases belong to a family of diseases that include light-chain (AL)-amyloid, nonamyloid fibrillary and immunotactoid glomerulonephritis, and cryoglobulinemic glomerulonephritis, in which monoclonal Ig or their subunits become deposited in kidney. In clinical and pathologic terms, light-, light- and heavy-, and heavy-chain deposition diseases essentially are similar and are characterized by prominent renal involvement with severe renal failure; extrarenal manifestations; diabetes-like nodular glomerulosclerosis; marked thickening of tubular basement membranes; and monotypic deposits of light chain, mostly , and/or heavy chain that feature a nonorganized granular, electron-dense appearance by electron microscopy. The most common cause is myeloma. Recent progress has been made in the understanding of the molecular pathomechanisms of Ig-chain deposition and extracellular matrix accumulation, which opens up new therapeutic avenues in addition to eradication of the Ig-secreting plasma cell clone. Because these diseases represent a model of glomerular and interstitial fibrosis that is induced by a single molecule species, a better understanding of their pathomechanisms may help to unravel the pathophysiology of kidney fibrosis and renal disease progression.

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