-Review-

Monoclonal Immunoglobulin Deposition Disease and Related Diseases

Go Kanzaki^1,2, Yusuke Okabayashi^1,2, Kiyotaka Nagahama³, Ryuji Ohashi⁴, Nobuo Tsuboi², Takashi Yokoo² and Akira Shimizu¹

¹Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan
²Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan
³Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan
⁴Department of Diagnostic Pathology, Nippon Medical School Hospital, Tokyo, Japan

Abnormal proliferation of plasma cells and some monoclonal B cells frequently cause the secretion of monoclonal immunoglobulins or immunoglobulin fragments into the serum, causing monoclonal gammopathy, which leads to various diseases including renal diseases. Therefore, monoclonal gammopathy is frequently associated with kidney diseases, including glomerular and tubulointerstitial diseases. Glomerular disease, with the deposition of monoclonal immunoglobulins or their components, includes monoclonal immunoglobulin deposition disease (MIDD), AL or AH amyloidosis, type I cryoglobulinemia, proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID), immunotactoid glomerulopathy, and fibrillary glomerulonephritis. In addition, tubulointerstitial diseases with the deposition of monoclonal immunoglobulins or their components are constituted by light chain (myeloma) cast nephropathy, light chain associated Fanconi's syndrome (light chain proximal [crystal] tubulopathy), and crystal-storing histiocytosis. In the present review article, we demonstrate the clinicopathological characteristics of MIDD, which is one of the representative diseases of plasma cell dyscrasias, and discuss various renal diseases with the deposition of monoclonal immunoglobulins or their components in glomeruli and the tubulointerstitium. We recommend that these renal diseases are arranged as one disease category, "renal diseases with deposition of monoclonal immunoglobulins or their components", in order to simplify the understanding of complicated diseases in plasma cell dysplasia.

J Nippon Med Sch 2019; 86: 2-9

Keywords
monoclonal immunoglobulin deposition disease (MIDD), light chain deposition disease (LCDD), glomerular deposition disease, monoclonal gammopathy of renal significance (MGRS), monoclonal gammopathy of undetermined significance (MGUS)

Correspondence to
Akira Shimizu, MD, PhD, Department of Analytic Human Pathology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8602, Japan
ashimizu@nms.ac.jp

Received, March 9, 2018
Accepted, August 16, 2018