SECTION 1: INTRODUCTION AND OVERVIEW 4 Introduction to Renal Pathology Robert B. Colvin, MD 24 Normal Kidney Structure Neeraja Kambham, MD and Anthony Chang, MD 36 Normal Kidney Development Sanjay Jain, MD, PhD 46 Kidney Biopsy Reports Surya V. Seshan, MD, Anthony Chang, MD, and Lynn D. Cornell, MD 48 Needle Biopsy: Evaluation for Adequacy Lynn D. Cornell, MD 52 Harmonized Nephropathology Terminology and Reporting Mark Haas, MD, PhD 54 Consensus Definitions of Light and Electron Microscopy Lesions Mark Haas, MD, PhD 58 Common Artifacts and Pitfalls Lynn D. Cornell, MD SECTION 2: GLOMERULAR DISEASES PODOCYTOPATHIES 66 Minimal Change Disease (Diffuse Podocytopathy) A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD 74 Classification of Focal Segmental Glomerulosclerosis A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD 78 Etiologic Classification of Focal Segmental Glomerulosclerosis Robert B.
Colvin, MD 80 Focal Segmental Glomerulosclerosis, Primary A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD 88 Focal Segmental Glomerulosclerosis, Adaptive (Secondary) A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD 94 Collapsing Glomerulopathy A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD MEMBRANOUS NEPHROPATHY AND VARIANTS 104 Introduction to Membranous Nephropathy Robert B. Colvin, MD and Anthony Chang, MD 112 Etiologies of Membranous Nephropathy Anthony Chang, MD and Robert B. Colvin, MD 114 Membranous Nephropathy Due to PLA2R1 Autoantibodies Anthony Chang, MD and Sanjeev Sethi, MD 120 Membranous Nephropathy Due to Non-PLA2R1 Autoantibodies Anthony Chang, MD and Sanjeev Sethi, MD 126 Membranous Nephropathy Associated With Systemic Disease Anthony Chang, MD 130 Membranous Nephropathy With Anti-TBM Antibodies Robert B. Colvin, MD 132 Podocyte Infolding Glomerulopathy Shane Meehan, MB, BCh IGA-RELATED GLOMERULONEPHRITIDES 134 Overview of IgA-Related Glomerular Diseases Shane Meehan, MB, BCh 138 IgA Nephropathy Lynn D. Cornell, MD, Robert B.
Colvin, MD, and Ian Roberts, MBChB, FRCPath 152 IgA Vasculitis (Henoch-Schönlein Purpura) Anthony Chang, MD and Ian Roberts, MBChB, FRCPath 158 IgA Acute Glomerulonephritis Associated With Staphylococcus aureus Robert B. Colvin, MD and Ian Roberts, MBChB, FRCPath 162 Hepatic Disease With IgA Deposition Robert B. Colvin, MD SLE AND RELATED AUTOANTIBODYMEDIATED DISEASES 164 Systemic Lupus Erythematosus Shane Meehan, MB, BCh, Robert B. Colvin, MD, and Ingeborg M. Bajema, MD, PhD 182 Mixed Connective Tissue Disease A. Brad Farris, III, MD, MBA 184 Idiopathic Nonlupus Full-House Nephropathy Ingeborg M. Bajema, MD, PhD and Emilie Rijnink, MD, PhD 186 Mixed Cryoglobulinemic Glomerulonephritis A. Brad Farris, III, MD, MBA and Charles E.
Alpers, MD ANTI-GBM DISEASES 192 Anti-GBM Glomerulonephritis Anthony Chang, MD 200 Atypical Anti-GBM Disease Lynn D. Cornell, MD COMPLEMENT-RELATED GLOMERULONEPHRITIDES 204 Classification of MPGN and Complement-Related Diseases Jean Hou, MD, Robert B. Colvin, MD, and Mark Haas, MD, PhD 210 Dense Deposit Disease Jean Hou, MD, Mark Haas, MD, PhD, and A. Brad Farris, III, MD, MBA 218 C3 Glomerulonephritis Jean Hou, MD, Lynn D. Cornell, MD, and Mark Haas, MD, PhD 226 C1q Nephropathy Neeraja Kambham, MD 232 C4 Glomerulopathy Sanjeev Sethi, MD and Lynn D. Cornell, MD 234 Immune Complex Membranoproliferative Glomerulonephritis Jean Hou, MD, A. Brad Farris, III, MD, MBA, and Mark Haas, MD, PhD MONOCLONAL IMMUNOGLOBULIN GLOMERULAR DISEASES 242 Introduction to Diseases With Monoclonal Immunoglobulin Deposits Robert B. Colvin, MD 246 Monoclonal Immunoglobulin Deposition Disease Lynn D.
Cornell, MD 254 Proliferative Glomerulonephritis With Monoclonal IgG Deposits Lynn D. Cornell, MD 258 Membranous Nephropathy With Masked IgG-Kappa Deposits Christopher P. Larsen, MD and Tiffany Caza, MD, PhD 262 Type I Cryoglobulinemic Glomerulonephritis Evan Farkash, MD, PhD and Robert B. Colvin, MD 266 Waldenström Macroglobulinemia Anthony Chang, MD 268 Crystalglobulin Glomerulonephritis Mariam Priya Alexander, MD 270 Light Chain Crystalline Podocytopathy Lynn D. Cornell, MD AMYLOIDOSIS 272 Amyloidosis Classification A. Brad Farris, III, MD, MBA and Sanjeev Sethi, MD 278 AL/AH Amyloidosis A. Brad Farris, III, MD, MBA and Sanjeev Sethi, MD 284 AA Amyloidosis A. Brad Farris, III, MD, MBA and Sanjeev Sethi, MD 288 AFib Amyloidosis A.
Brad Farris, III, MD, MBA and Sanjeev Sethi, MD 290 AGel Amyloidosis Sanjeev Sethi, MD 292 ALECT2 Amyloidosis Christopher P. Larsen, MD, Sanjeev Sethi, MD, and Tiffany Caza, MD, PhD 294 AApoAI Amyloidosis A. Brad Farris, III, MD, MBA and Sanjeev Sethi, MD 295 AApoAII Amyloidosis Robert B. Colvin, MD and Sanjeev Sethi, MD 296 AApoAIV Amyloidosis Sanjeev Sethi, MD 297 AApoCII Amyloidosis Sanjeev Sethi, MD 298 ATTR Amyloidosis A. Brad Farris, III, MD, MBA and Sanjeev Sethi, MD IDIOPATHIC FIBRILLARY GLOMERULOPATHIES 300 Diseases With Organized Deposits Robert B. Colvin, MD 304 Fibrillary Glomerulopathy Anthony Chang, MD and Mariam Priya Alexander, MD 310 Immunotactoid Glomerulopathy Anthony Chang, MD and Charles E. Alpers, MD 314 Fibronectin Glomerulopathy Neeraja Kambham, MD DIABETIC AND RELATED RENAL DISEASES 318 Diabetic Nephropathy Neeraja Kambham, MD and Ian Roberts, MBChB, FRCPath 328 Metabolic Syndrome/Obesity-Related Glomerular Disease Shane Meehan, MB, BCh 332 Idiopathic Nodular Glomerulopathy A. Brad Farris, III, MD, MBA and Ian Roberts, MBChB, FRCPath INFECTION-RELATED GLOMERULAR DISEASES 336 Overview of Infection-Related Glomerular Disease Shane Meehan, MB, BCh 340 Acute Poststreptococcal Glomerulonephritis A.
Brad Farris, III, MD, MBA 348 Postinfectious Glomerulonephritis, Nonstreptococcal A. Brad Farris, III, MD, MBA 354 Glomerulonephritis of Chronic Infection, Including Shunt Nephritis A. Brad Farris, III, MD, MBA 356 Endocarditis A. Brad Farris, III, MD, MBA and Christie L. Boils, MD 360 Syphilis Shane Meehan, MB, BCh 362 Lyme Disease Neeraja Kambham, MD 364 COVID-19 Nephropathies Lynn D. Cornell, MD, Mariam Priya Alexander, MD, and Robert B. Colvin, MD 372 Hepatitis B Virus Anthony Chang, MD 376 Hepatitis C Virus Anthony Chang, MD and Charles E. Alpers, MD 382 HIV-Associated Nephropathy A.
Brad Farris, III, MD, MBA 388 Miscellaneous HIV-Associated Renal Diseases A. Brad Farris, III, MD, MBA 394 Schistosomiasis Neeraja Kambham, MD 396 Filariasis Christie L. Boils, MD 397 Leishmaniasis Kerstin Amann, MD and Robert B. Colvin, MD DRUG-INDUCED GLOMERULAR DISEASES 398 Drug-Induced Minimal Change Disease Anthony Chang, MD and Astrid Weins, MD, PhD 400 Bisphosphonate-Induced Collapsing Glomerulopathy A. Brad Farris, III, MD, MBA and Astrid Weins, MD, PhD 401 Chloroquine Toxicity Shane Meehan, MB, BCh 402 Antihepatitis C Virus Drugs Ivy A. Rosales, MD 406 Anti-EGFR Drugs Kammi J. Henriksen, MD GENETIC GLOMERULAR DISEASES 408 Classification of Glomerular Genetic Diseases Sanjay Jain, MD, PhD 410 Genetic Causes of Nephrotic Syndrome Joseph P. Gaut, MD, PhD GENETIC DISEASES OF GBM COLLAGEN 414 Alport Syndrome Joseph P.
Gaut, MD, PhD, Helen Liapis, MD, and Robert B. Colvin, MD 426 Thin Basement Membrane Disease Joseph P. Gaut, MD, PhD and Helen Liapis, MD GENETIC PODOCYTE DISEASES 428 Congenital Nephrotic Syndrome of Finnish Type Joseph P. Gaut, MD, PhD and Helen Liapis, MD 432 Pierson Syndrome Nidia Messias, MD, Joseph P. Gaut, MD, PhD, and Helen Liapis, MD 434 Galloway-Mowat Syndrome Carrie L. Phillips, MD and Stephen M. Bonsib, MD 436 Denys-Drash Syndrome Kammi J. Henriksen, MD and Stephen M.
Bonsib, MD 440 Frasier Syndrome Carrie L. Phillips, MD and Stephen M. Bonsib, MD 442 Podocin Deficiency Joseph P. Gaut, MD, PhD and Helen Liapis, MD 444 a-Actinin-4 Deficiency Joseph P. Gaut, MD, PhD and Helen Liapis, MD 446 Autosomal Dominant FSGS Due to INF2 Mutations Robert B. Colvin, MD 448 APOL1-Related Glomerular Disease Christopher P. Larsen, MD and Tiffany Caza, MD, PhD 452 Schimke Immunoosseous Dysplasia Shane Meehan, MB, BCh GENETIC STORAGE AND LIPID DISEASES 454 Fabry Disease Robert B. Colvin, MD and Laura Barisoni, MD 462 Alagille Syndrome Anthony Chang, MD 464 Lecithin Cholesterol Acyltransferase Deficiency Carrie L.
Phillips, MD and Stephen M. Bonsib, MD 468 APOE Lipoprotein Glomerulopathy Joseph P. Gaut, MD, PhD and Helen Liapis, MD 470 Type III Hyperlipoproteinemia Nidia Messias, MD, Joseph P. Gaut, MD, PhD, and Helen Liapis, MD 472 I-Cell Disease (Mucolipidosis II) Carrie L. Phillips, MD and Stephen M. Bonsib, MD 474 Gaucher Glomerulopathy A. Brad Farris, III, MD, MBA OTHER GENETIC GLOMERULAR DISEASES 476 Type III Collagen Glomerulopathy Nidia Messias, MD, L. Nicholas Cossey, MD, and Helen Liapis, MD 480 Nail-Patella Syndrome Nidia Messias, MD and Helen Liapis, MD 482 Glomerulopathy of Hereditary Multiple Exostoses Robert B.
Colvin, MD and Ian Roberts, MBChB, FRCPath MISCELLANEOUS GLOMERULAR DISEASES 484 Cryofibrinogenic Glomerulopathy Lynn D. Cornell, MD and Mariam Priya Alexander, MD 488 IgM Nephropathy Neeraja Kambham, MD 490 Kidney Disease Associated With COVID-19 Immunization Astrid Weins, MD, PhD and Robert B. Colvin, MD 494 Renal Disease in Rheumatoid Arthritis Neeraja Kambham, MD 500 Hemophagocytic Glomerulopathy Lynn D. Cornell, MD 504 Coenzyme-Q Nephropathies Laura Barisoni, MD 506 Hepatic Glomerulosclerosis Robert B. Colvin, MD 507 Intravascular Large B-Cell Lymphoma Anthony Chang, MD SECTION 3: VASCULAR DISEASES 510 Overview and Classification of Systemic Vasculitides Surya V. Seshan, MD and Ingeborg M. Bajema, MD, PhD ANCA DISEASES 520 ANCA-Related Glomerulonephritis Surya V. Seshan, MD and Ingeborg M.
Bajema, MD, PhD 526 Microscopic Polyangiitis Surya V. Seshan, MD and Ingeborg M. Bajema, MD, PhD 534 Granulomatosis With Polyangiitis Surya V. Seshan, MD and Ingeborg M. Bajema, MD, PhD 544 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Surya V. Seshan, MD and Ingeborg M. Bajema, MD, PhD 550 Drug-Induced ANCA Vasculitis Surya V. Seshan, MD and Ingeborg M.
Bajema, MD, PhD NON-ANCA DISEASES 554 P.