Gerald B. Appel, MD (Director)
Professor of Medicine
Education & Training
- Improving Clinical Trials for Anticomplement Therapies in Complement-Mediated Glomerulopathies: Report of a Scientific Workshop Sponsored by the National Kidney Foundation September 27, 2021Blocking the complement system as a therapeutic strategy has been proposed for numerous glomerular diseases but presents myriad questions and challenges, not the least of which is demonstrating efficacy and safety. In light of these potential issues and because there are an increasing number of anticomplement therapy trials either planned or under way, the National […]
- Anti-neutrophil cytoplasmic antibody associated glomerulonephritis complicating treatment with hydralazine April 15, 2021Hydralazine, a widely used therapy for hypertension and heart failure, can elicit autoimmune disease, including anti-neutrophil cytoplasmic antibody associated glomerulonephritis (ANCA-GN). We identified 80 cases of ANCA-GN complicating treatment with hydralazine, accounting for 4.3% (80/1858 biopsies) of ANCA-GN diagnosed between 2006 and 2019. Over three-fourths of patients were on hydralazine for at least one year, […]
- Study Design and Baseline Characteristics of the CARDINAL Trial: A Phase 3 Study of Bardoxolone Methyl in Patients with Alport Syndrome March 31, 2021INTRODUCTION: Alport syndrome is a rare genetic disorder that affects as many as 60,000 persons in the USA and a total of 103,000 persons (
- Association of Treatment Effects on Early Change in Urine Protein and Treatment Effects on GFR Slope in IgA Nephropathy: An Individual Participant Meta-analysis March 29, 2021CONCLUSIONS: These results provide new evidence supporting that early reduction in proteinuria can be used as a surrogate end point for studies of chronic kidney disease progression in IgAN.
- Familial Fibrillary Glomerulonephritis in Living Related Kidney Transplantation January 11, 2021No abstract
- Concurrent Anti-Glomerular Basement Membrane Antibody Disease and Membranous Nephropathy: A Case Series January 9, 2021CONCLUSIONS: In this case series, the presentation of concurrent anti-GBM disease and MN was characterized by rapidly progressive glomerulonephritis and poor kidney outcomes. These findings suggest possible value from earlier diagnosis and the need for identification of more effective treatment regimens.
- Mycophenolate Mofetil Treatment of C3 Glomerulopathy August 26, 2020No abstract
- Standardized reporting of monoclonal immunoglobulin-associated renal diseases: recommendations from a Mayo Clinic/Renal Pathology Society Working Group July 26, 2020No abstract
- Association of HLA Typing and Alloimmunity With Posttransplantation Membranous Nephropathy: A Multicenter Case Series May 4, 2020CONCLUSIONS: De novo and recurrent MN likely represent separate diseases. De novo MN is associated with humoral alloimmunity and guarded outcome. Potential predisposing factors for recurrent MN include recipients who are older, recipient HLA-A3 antigen, steroid-free immunosuppressive regimen, and living related donor kidney.
- How COVID-19 Has Changed the Management of Glomerular Diseases April 26, 2020No abstract
- Pilot Study of Return of Genetic Results to Patients in Adult Nephrology April 18, 2020CONCLUSIONS: Developing the infrastructure to support return of genetic results in nephrology was resource-intensive, but presented potential opportunities for improving patient care.
- The genetic architecture of membranous nephropathy and its potential to improve non-invasive diagnosis April 2, 2020Membranous Nephropathy (MN) is a rare autoimmune cause of kidney failure. Here we report a genome-wide association study (GWAS) for primary MN in 3,782 cases and 9,038 controls of East Asian and European ancestries. We discover two previously unreported loci, NFKB1 (rs230540, OR = 1.25, P = 3.4 × 10^(-12)) and IRF4 (rs9405192, OR = […]
- Antigens, antibodies, and membranous nephropathy: a decade of progress January 6, 2020Membranous nephropathy (MN) is either primary or associated with various etiologies, each with unique glomerular antigens. The discovery of the phospholipase A2 receptor (PLA2R) antigen in primary MN revolutionized our understanding of MN and led to major clinical progress. Other recently discovered antigens in MN include the THSD7A antigen and exostosin. Sethi et al. have […]
- Clinicopathologic Assessment of Monoclonal Immunoglobulin-associated Renal Disease in the Kidney Allograft: A Retrospective Study and Review of the Literature October 22, 2019CONCLUSIONS: MIgARD in the kidney allograft is associated with poor prognosis. While posttransplant PGNMID can change its apparent clonality by immunofluorescence supporting oligoclonal immune responses, the presence of deposit substructure is an important indicator of underlying hematologic neoplasm. Non-PGNMID are often associated with hematologic neoplasms and varied prognosis.
- Contributions of Rare Gene Variants to Familial and Sporadic FSGS July 17, 2019CONCLUSIONS: Although our analysis validated many known FSGS-causing genes, we detected a nontrivial number of purported "disease-causing" variants in controls, implying that filtering is inadequate to allow clinical diagnosis and decision making. Genetic diagnosis in patients with FSGS is complicated by the nontrivial rate of variants in known FSGS genes among people without kidney disease.