title: Paraprotein-Related Kidney Disease: Kidney Injury from Paraproteins-What Determines the Site of Injury?
abstract: Disorders of plasma and B cells leading to paraproteinemias are associated with a variety of renal diseases. Understanding the mechanisms of injury and associated nephropathies provides a framework that aids clinicians in prompt diagnosis and appropriate adjunctive treatment of these disorders. Glomerular diseases that may be associated with paraproteinemias include amyloid deposition, monoclonal Ig deposition disease, proliferative GN with monoclonal Ig deposits, C3 glomerulopathy caused by alterations in the complement pathway, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemia. Tubular lesions include the classic Fanconi syndrome, light-chain proximal tubulopathy, interstitial fibrosis, and cast nephropathy. These paraproteinemic renal diseases are distinct in their pathogenesis as well as their urinary and kidney biopsy findings. Renal pathology is usually initiated by deposition and direct involvement of the intact monoclonal Ig or Ig fragments with resident cells of the nephron. Our review summarizes current insights into the underlying molecular pathogenesis of these interesting kidney lesions.
authors.affiliation: Division of Nephrology, Wayne State University School of Medicine, Detroit, Michigan.
authors.forename: Mona
authors.initials: M
authors.lastname: Doshi
authors.affiliation: Section of Nephrology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Amit
authors.initials: A
authors.lastname: Lahoti
authors.affiliation: Section of Nephrology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Farhad R
authors.initials: FR
authors.lastname: Danesh
authors.affiliation: Department of Medicine, Southeast Louisiana Veterans Health Care System, New Orleans, Louisiana.|Department of Medicine, Tulane University Medical School, New Orleans, Louisiana.
authors.forename: Vecihi
authors.initials: V
authors.lastname: Batuman
authors.affiliation: Department of Medicine, Nephrology Research and Training Center and.|Department of Cell, Developmental and Integrative Biology, University of Alabama at Birmingham, Birmingham, Alabama; and.|Department of Medicine, Veterans Affairs Medical Center, Birmingham, Alabama.
authors.forename: Paul W
authors.initials: PW
authors.lastname: Sanders
mesh_terms: D000686:Amyloidosis / Q000150:complications*; D005921:Glomerulonephritis / Q000209:etiology / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007674:Kidney Diseases / Q000209:etiology* / Q000473:pathology*; D007678:Kidney Glomerulus; D007686:Kidney Tubules, Distal; D007687:Kidney Tubules, Proximal; D010265:Paraproteinemias / Q000150:complications*; D010266:Paraproteins / Q000378:metabolism*
chemical_list: D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D010266:Paraproteins
keywords: Kidney Diseases; Myeloma Proteins; Paraproteins; amyloidosis; glomerular disease; immunoglobulin free light chains; immunoglobulins; kidney; multiple myeloma; multiple myeloma M-proteins; paraproteins; tubular epithelium