IgG4-related kidney disease: Clinicopathologic features, differential diagnosis, and mimics. 2024

Sarwat I Gilani, and Alessia Buglioni, and Lynn D Cornell
Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio, TX, USA.

IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.

UI MeSH Term Description Entries
D007074 Immunoglobulin G The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B. Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D007668 Kidney Body organ that filters blood for the secretion of URINE and that regulates ion concentrations. Kidneys
D009395 Nephritis, Interstitial Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction. Interstitial Nephritis,Nephritis, Tubulointerstitial,Tubulointerstitial Nephritis,Interstitial Nephritides,Nephritides, Interstitial,Nephritides, Tubulointerstitial,Tubulointerstitial Nephritides
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000077733 Immunoglobulin G4-Related Disease A spectrum of systemic autoimmune diseases in which IMMUNOGLOBULIN G4 plays a pathophysiologic role. It can affect multiple organs in highly variable presentations, characterized by inflammatory lesions composed of IgG4-positive PLASMA CELLS, further infiltrated by T helper cells (T-LYMPHOCYTES, HELPER-INDUCER) when linked to progressive FIBROSIS and eventual organ damage. IgG4-Related Kidney Disease,IgG4-Related Sclerosing Disease,Immunoglobulin G4-Related Kidney Disease,Immunoglobulin G4-Related Sclerosing Disease,IgG4 Related Systemic Disease,IgG4-Associated Autoimmune Disease,IgG4-RD,IgG4-Related Disease,Autoimmune Disease, IgG4-Associated,IgG4 Associated Autoimmune Disease,IgG4 Related Disease,IgG4 Related Kidney Disease,IgG4 Related Sclerosing Disease,IgG4-Associated Autoimmune Diseases,IgG4-Related Diseases,IgG4-Related Kidney Diseases,IgG4-Related Sclerosing Diseases,Immunoglobulin G4 Related Disease,Immunoglobulin G4 Related Kidney Disease,Immunoglobulin G4 Related Sclerosing Disease,Immunoglobulin G4-Related Diseases,Kidney Disease, IgG4-Related,Sclerosing Disease, IgG4-Related
D015433 Glomerulonephritis, Membranous A type of glomerulonephritis that is characterized by the accumulation of immune deposits (COMPLEMENT MEMBRANE ATTACK COMPLEX) on the outer aspect of the GLOMERULAR BASEMENT MEMBRANE. It progresses from subepithelial dense deposits, to basement membrane reaction and eventual thickening of the basement membrane. Heymann Nephritis,Membranous Glomerulopathy,Nephropathy, Membranous,Extramembranous Glomerulopathy,Idiopathic Membranous Glomerulonephritis,Idiopathic Membranous Nephropathy,Membranous Glomerulonephropathy,Membranous Nephropathy,Glomerulonephritides, Idiopathic Membranous,Glomerulonephritides, Membranous,Glomerulonephritis, Idiopathic Membranous,Glomerulonephropathy, Membranous,Glomerulopathy, Extramembranous,Glomerulopathy, Membranous,Idiopathic Membranous Glomerulonephritides,Membranous Glomerulonephritides,Membranous Glomerulonephritides, Idiopathic,Membranous Glomerulonephritis,Membranous Glomerulonephritis, Idiopathic,Membranous Nephropathy, Idiopathic,Nephritis, Heymann,Nephropathy, Idiopathic Membranous

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