9 - Monoclonal immunoglobulin deposition disease

Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of monoclonal light and/or heavy chains within glomerular, tubular and vessel wall basement membranes. Three subtypes of MIDD have been described and they are subdivided according to the composition of monoclonal protein deposited. The most common form of MIDD is light chain deposition disease (LCDD), where either kappa or lambda light chains are deposited in tubular, glomerular and vessel wall basement membranes. Much less common than LCDD are heavy chain deposition disease (HCDD) and light and heavy chain deposition disease (LHCDD), which show basement membrane accumulation of a monoclonal immunoglobulin heavy chain, either with a corresponding monoclonal light chain (LHCDD) or without an accompanying light chain (HCDD). MIDD is relatively uncommon and occurs at approximately half the rate of amyloidosis. Roughly half of patients diagnosed with MIDD will have myeloma. Although MIDD can present as a multisystem disease, renal manifestations typically predominate. More than 83% of the patients reported with MIDD and kidney disease had renal insufficiency. Proteinuria is present in almost all patients with MIDD and in about 40%–50% of patients will have nephrotic range proteinuria. Optimal treatment for MIDD remains to be determined, but the use of proteasome inhibitors and autologous stem cell transplantation has shown encouraging results with regards to both hematologic remission and improvement in renal function.