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205. Contributors

Author

Abudayyeh, Ala, Adrogué, Horacio J., Ambruso, Sophia L., Anand, Shuchi, Bansal, Amar D., Barratt, Jonathan, Bazeley, Jonathan W., Berns, Jeffrey S., Bjornstad, Petter, Bobart, Shane A., Bomback, Andrew S., Braun, Daniela A., Brewster, Ursula C., Cattran, Daniel C., Chandraker, Anil, Chandran, Sindhu, Chapman, Arlene B., Cherney, David Z., Cohen, Debbie L., Cook, Jared, Cortazar, Frank B., Dad, Taimur, D’Agati, Vivette D., De Vriese, An S., Derebail, Vimal K., DuBose, Thomas D., Jr., Dubrofsky, Lisa, Emmett, Michael, Evenepoel, Pieter, Fairhead, Todd, Ferrey, Antoney J., Fervenza, Fernando C., Finkel, Kevin W., Flood, Ryan P., Födinger, Manuela, Freedman, Barry I., Garcia, Pablo, Garratt, Joanie M., Gelfand, Samantha L., Gilbert, Scott J., Greenberg, Arthur, Gregory, Martin C., Gunning, Samantha, Herlitz, Leal, Hildebrandt, Friedhelm, Hladik, Gerald A., Hladunewich, Michelle A., Hoenig, Melanie P., Hogan, Jonathan, Ikizler, T. Alp, Inker, Lesley A., Ison, Michael G., Juncos, Luis A., Kain, Renate, Kala, Jaya, Kalantar-Zadeh, Kamyar, Kendrick, Jessica, Knauf, Felix, Knoll, Greg, Kopp, Jeffrey B., Kovesdy, Csaba P., Koyner, Jay L., Kruzel-Davila, Etty, Levey, Andrew S., Li, Jian, Linas, Stuart L., Lopez-Ruiz, Arnaldo, Luciano, Randy L., Luyckx, Valerie A., Ma, Lijun, Macedo, Etienne, Madias, Nicolaos E., Mehta, Ankit N., Mehta, Ravindra L., Misra, Madhukar, Morgan, Matthew A., Nast, Cynthia C., Ngamvichchukorn, Tanun, Nolin, Thomas D., O’Hare, Ann, Olaniran, Kabir O., Pantel, Austin R., Peixoto, Aldo J., Perazella, Mark A., Perera, Dinushi S., Perl, Jeffrey, Pfau, Anja, Provenzano, Laura Ferreira, Quarles, L. Darryl, Radhakrishnan, Jai, Rahbari-Oskoui, Frederic F., Rhee, Connie, Rizk, Dana V., Rosenberg, Avi Z., Rosner, Mitchell H., Sahinoz, Melis, Sampogna, Rosemary V., Sanders, Paul W., Sarnak, Mark J., Scheinman, Steven J., Schell, Jane O., Schnaper, H. William, Schrauben, Sarah, Schreiber, Brittany L., Sethi, Sanjeev, Shah, Nikhil, Shen, Wen, Shirali, Anushree C., Sise, Meghan E., Soi, Vivek, Strohbehn, Ian A., Sunder-Plassmann, Gere, Sutherland, Richard W., Sy, John E., Szerlip, Harold M., Tangren, Jessica, Tangri, Navdeep, Testani, Jeffrey M., Thurman, Joshua M., Townsend, Raymond R., Trachtman, Howard, Turner, Jeffrey M., Velez, Juan Carlos Q., Verbalis, Joseph G., Vincenti, Flavio G., Vivarelli, Marina, Warady, Bradley A., Weidemann, Darcy K., Weiner, Daniel E., Wilcox, Christopher S., Wish, Jay B., Yee, Jerry, and Yeo, See Cheng

Published
2023
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206. Circulating Markers of Vascular Injury and Angiogenesis in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Author

Monach, Paul A., Tomasson, Gunnar, Specks, Ulrich, Stone, John H., Cuthbertson, David, Krischer, Jeffrey, Ding, Linna, Fervenza, Fernando C., Fessler, Barri J., Hoffman, Gary S., Ikle, David, Kallenberg, Cees G. M., Langford, Carol A., Mueller, Mark, Seo, Philip, St.Clair, William E., Spiera, Robert, Tchao, Nadia, Ytterberg, Steven R., Gu, Yi-Zhong, Snyder, Ronald D., and Merkel, Peter A.

Published
2011
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209. Pirfenidone for Diabetic Nephropathy

Author

Sharma, Kumar, Ix, Joachim H., Mathew, Anna V., Cho, Monique, Pflueger, Axel, Dunn, Stephen R., Francos, Barbara, Sharma, Shoba, Falkner, Bonita, McGowan, Tracy A., Donohue, Michael, RamachandraRao, Satish, Xu, Ronghui, Fervenza, Fernando C., and Kopp, Jeffrey B.

Published
2011
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210. Long-term outcomes of patients with light chain amyloidosis (AL) after renal transplantation with or without stem cell transplantation

Author

Herrmann, Sandra M.S., Gertz, Morie A., Stegall, Mark D., Dispenzieri, Angela, Cosio, Fernando C., Kumar, Shaji, Lacy, Martha Q., Dean, Patrick G., Prieto, Mikel, Zeldenrust, Steven R., Buadi, Francis K., Russell, Stephen J., Nyberg, Scott L., Hayman, Suzanne R., Dingli, David, Fervenza, Fernando C., and Leung, Nelson

Published
2011
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213. Determination of Relapse Risk By Complement Gene Variants after Eculizumab Discontinuation in Complement-Mediated Thrombotic Microangiopathy: A Retrospective Review

Author

Acosta-Medina, Aldo A., primary, Moyer, Ann M., additional, Go, Ronald S., additional, Willrich, Maria Alice V., additional, Alkhateeb, Hassan B., additional, Bourlon De Los Rios, Christianne, additional, Chen, Dong, additional, Fervenza, Fernando C., additional, Heikal, Nahla M., additional, Leung, Nelson, additional, Marshall, Ariela L., additional, Tran, Cheryl L., additional, Winters, Jeffrey L., additional, and Sridharan, Meera, additional

Published
2020
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216. Recurrence of DNAJB9-Positive Fibrillary Glomerulonephritis After Kidney Transplantation: A Case Series

Author

El Ters, Mireille, primary, Bobart, Shane A., additional, Cornell, Lynn D., additional, Leung, Nelson, additional, Bentall, Andrew, additional, Sethi, Sanjeev, additional, Fidler, Mary, additional, Grande, Joseph, additional, Hernandez, Loren Herrera, additional, Cosio, Fernando G., additional, Zand, Ladan, additional, Amer, Hatem, additional, Fervenza, Fernando C., additional, Nasr, Samih H., additional, and Alexander, Mariam P., additional

Published
2020
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217. Longitudinal Changes in Health-Related Quality of Life in Primary Glomerular Disease: Results From the CureGN Study

Author

Murphy, Shannon L., primary, Mahan, John D., additional, Troost, Jonathan P., additional, Srivastava, Tarak, additional, Kogon, Amy J., additional, Cai, Yi, additional, Davis, T. Keefe, additional, Fernandez, Hilda, additional, Fornoni, Alessia, additional, Gbadegesin, Rasheed A., additional, Herreshoff, Emily, additional, Canetta, Pietro A., additional, Nachman, Patrick H., additional, Reeve, Bryce B., additional, Selewski, David T., additional, Sethna, Christine B., additional, Wang, Chia-shi, additional, Bartosh, Sharon M., additional, Gipson, Debbie S., additional, Tuttle, Katherine R., additional, Gharavi, Ali, additional, Ahn, Wooin, additional, Appel, Gerald B., additional, Avasare, Rupali S., additional, Babayev, Revekka, additional, Batal, Ibrahim, additional, Bomback, Andrew S., additional, Brown, Eric, additional, Campenot, Eric S., additional, Canetta, Pietro, additional, Chan, Brenda, additional, D’Agati, Vivette D., additional, Foroncewicz, Bartosz, additional, Ghiggeri, Gian Marco, additional, Hines, William H., additional, Jain, Namrata G., additional, Kiryluk, Krzysztof, additional, Lin, Fangming, additional, Lugani, Francesca, additional, Marasa, Maddalena, additional, Markowitz, Glen, additional, Mohan, Sumit, additional, Mucha, Krzysztof, additional, Nickolas, Thomas L., additional, Radhakrishnan, Jai, additional, Rao, Maya K., additional, Regunathan-Shenk, Renu, additional, Sanna-Cherchi, Simone, additional, Santoriello, Dominick, additional, Stokes, Michael B., additional, Yu, Natalie, additional, Valeri, Anthony M., additional, Zviti, Ronald, additional, Greenbaum, Larry A., additional, Smoyer, William E., additional, Al-Uzri, Amira, additional, Ashoor, Isa, additional, Aviles, Diego, additional, Baracco, Rossana, additional, Barcia, John, additional, Bartosh, Sharon, additional, Belsha, Craig, additional, Braun, Michael C., additional, Chishti, Aftab, additional, Claes, Donna, additional, Cramer, Carl, additional, Davis, Keefe, additional, Erkan, Elif, additional, Feig, Daniel, additional, Freundlich, Michael, additional, Hanna, Melisha, additional, Hidalgo, Guillermo, additional, Jain, Amrish, additional, Khalid, Myda, additional, Kallash, Mahmoud, additional, Lane, Jerome C., additional, Mahan, John, additional, Mathews, Nisha, additional, Nester, Carla, additional, Pan, Cynthia, additional, Patel, Hiren, additional, Revell, Adelaide, additional, Sreedharan, Rajasree, additional, Steinke, Julia, additional, Wenderfer, Scott E., additional, Wong, Craig S., additional, Falk, Ronald, additional, Cook, William, additional, Derebail, Vimal, additional, Fogo, Agnes, additional, Gasim, Adil, additional, Gehr, Todd, additional, Harris, Raymond, additional, Kidd, Jason, additional, Laurin, Louis-Philippe, additional, Pendergraft, Will, additional, Pichette, Vincent, additional, Powell, Thomas Brian, additional, Renfrow, Matthew B., additional, Royal, Virginie, additional, Holzman, Lawrence B., additional, Adler, Sharon, additional, Alpers, Charles, additional, Matar, Raed Bou, additional, Brown, Elizabeth, additional, Choi, Michael, additional, Dell, Katherine M., additional, Dukkipati, Ram, additional, Fervenza, Fernando C., additional, Gadegbeku, Crystal, additional, Gipson, Patrick, additional, Hasely, Leah, additional, Hingorani, Sangeeta, additional, Hladunewich, Michelle A., additional, Hogan, Jonathan, additional, Jefferson, J. Ashley, additional, Jhaveri, Kenar, additional, Johnstone, Duncan B., additional, Kaskel, Frederick, additional, Kogan, Amy, additional, Kopp, Jeffrey, additional, Lemley, Kevin V., additional, Dieguez, Laura Malaga-, additional, Meyers, Kevin, additional, Neu, Alicia, additional, O'Shaughnessy, Michelle Marie, additional, O’Toole, John F., additional, Parekh, Rulan, additional, Reich, Heather, additional, Reidy, Kimberly, additional, Rondon, Helbert, additional, Sambandam, Kamalanathan K., additional, Sedor, John R., additional, Schelling, Jeffrey, additional, Sperati, John C., additional, Swiatecka-Urban, Agnes, additional, Trachtman, Howard, additional, Weisstuch, Joseph, additional, Zhdanova, Olga, additional, Gillespie, Brenda, additional, Kretzler, Matthias, additional, Robinson, Bruce M., additional, Mariani, Laura, additional, Wladkowski, Matthew, additional, and Guay-Woodford, Lisa M., additional

Published
2020
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219. PEXIVAS

Author

De Vriese, An S., primary and Fervenza, Fernando C., additional

Published
2020
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221. Efficacy of Rituximab and Plasma Exchange in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis with Severe Kidney Disease

Author

Casal Moura, Marta, primary, Irazabal, Maria V., additional, Eirin, Alfonso, additional, Zand, Ladan, additional, Sethi, Sanjeev, additional, Borah, Bijan J., additional, Winters, Jeffrey L., additional, Moriarty, James P., additional, Cartin-Ceba, Rodrigo, additional, Berti, Alvise, additional, Baqir, Misbah, additional, Thompson, Gwen E., additional, Makol, Ashima, additional, Warrington, Kenneth J., additional, Thao, Viengneesee, additional, Specks, Ulrich, additional, and Fervenza, Fernando C., additional

Published
2020
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223. Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Author

Delbarba, Elisa, primary, Marasa, Maddalena, additional, Canetta, Pietro A., additional, Piva, Stacy E., additional, Chatterjee, Debanjana, additional, Kil, Byum Hee, additional, Mu, Xueru, additional, Gibson, Keisha L., additional, Hladunewich, Michelle A., additional, Hogan, Jonathan J., additional, Julian, Bruce A., additional, Kidd, Jason M., additional, Laurin, Louis-Philippe, additional, Nachman, Patrick H., additional, Rheault, Michelle N., additional, Rizk, Dana V., additional, Sanghani, Neil S., additional, Trachtman, Howard, additional, Wenderfer, Scott E., additional, Gharavi, Ali G., additional, Bomback, Andrew S., additional, Ahn, Wooin, additional, Appel, Gerald B., additional, Babayev, Revekka, additional, Batal, Ibrahim, additional, Brown, Eric, additional, Campenot, Eric S., additional, Canetta, Pietro, additional, Chan, Brenda, additional, D’Agati, Vivette D., additional, Delbarba, Elisa, additional, Fernandez, Hilda, additional, Foroncewicz, Bartosz, additional, Ghiggeri, Gian Marco, additional, Hines, William H., additional, Jain, Namrata G., additional, Kiryluk, Krzysztof, additional, Lau, Wai L., additional, Lin, Fangming, additional, Lugani, Francesca, additional, Markowitz, Glen, additional, Mohan, Sumit, additional, Mucha, Krzysztof, additional, Nickolas, Thomas L., additional, Piva, Stacy, additional, Radhakrishnan, Jai, additional, Rao, Maya K., additional, Sanna-Cherchi, Simone, additional, Santoriello, Dominick, additional, Stokes, Michael B., additional, Yu, Natalie, additional, Valeri, Anthony M., additional, Zviti, Ronald, additional, Greenbaum, Larry A., additional, Smoyer, William E., additional, Al-Uzri, Amira, additional, Ashoor, Isa, additional, Aviles, Diego, additional, Baracco, Rossana, additional, Barcia, John, additional, Bartosh, Sharon, additional, Belsha, Craig, additional, Bowers, Corinna, additional, Braun, Michael C., additional, Chishti, Aftab, additional, Claes, Donna, additional, Cramer, Carl, additional, Davis, Keefe, additional, Erkan, Elif, additional, Feig, Daniel, additional, Freundlich, Michael, additional, Gbadegesin, Rasheed, additional, Hanna, Melisha, additional, Hidalgo, Guillermo, additional, Hunley, Tracy E., additional, Jain, Amrish, additional, Kallash, Mahmoud, additional, Khalid, Myda, additional, Klein, Jon B., additional, Lane, Jerome C., additional, Mahan, John, additional, Mathews, Nisha, additional, Nester, Carla, additional, Pan, Cynthia, additional, Patterson, Larry, additional, Patel, Hiren, additional, Revell, Adelaide, additional, Silva, Cynthia, additional, Sreedharan, Rajasree, additional, Srivastava, Tarak, additional, Steinke, Julia, additional, Twombley, Katherine, additional, Vasylyeva, Tetyana L., additional, Weaver, Donald J., additional, Wong, Craig S., additional, Almaani, Salem, additional, Ayoub, Isabelle, additional, Budisavljevic, Milos, additional, Derebail, Vimal, additional, Fatima, Huma, additional, Falk, Ronald, additional, Fogo, Agnes, additional, Gehr, Todd, additional, Gibson, Keisha, additional, Glenn, Dorey, additional, Harris, Raymond, additional, Hogan, Susan, additional, Jain, Koyal, additional, Jennette, J. Charles, additional, Julian, Bruce, additional, Kidd, Jason, additional, Massey, H. Davis, additional, Mottl, Amy, additional, Nachman, Patrick, additional, Nadasdy, Tibor, additional, Novak, Jan, additional, Parikh, Samir, additional, Pichette, Vincent, additional, Poulton, Caroline, additional, Powell, Thomas Brian, additional, Renfrow, Matthew, additional, Rizk, Dana, additional, Rovin, Brad, additional, Royal, Virginie, additional, Saha, Manish, additional, Sanghani, Neil, additional, Self, Sally, additional, Adler, Sharon, additional, Alpers, Charles, additional, Matar, Raed Bou, additional, Brown, Elizabeth, additional, Cattran, Daniel, additional, Choi, Michael, additional, Dell, Katherine M., additional, Dukkipati, Ram, additional, Fervenza, Fernando C., additional, Fornoni, Alessia, additional, Gadegbeku, Crystal, additional, Gipson, Patrick, additional, Hasely, Leah, additional, Hingorani, Sangeeta, additional, Hladunewich, Michelle, additional, Hogan, Jonathan, additional, Holzman, Lawrence B., additional, Jefferson, J. Ashley, additional, Jhaveri, Kenar, additional, Johnstone, Duncan B., additional, Kaskel, Frederick, additional, Kogan, Amy, additional, Kopp, Jeffrey, additional, Lafayette, Richard, additional, Lemley, Kevin V., additional, Malaga-Dieguez, Laura, additional, Meyers, Kevin, additional, Neu, Alicia, additional, O’Shaughnessy, Michelle Marie, additional, O’Toole, John F., additional, Parekh, Rulan, additional, Reich, Heather, additional, Reidy, Kimberly, additional, Rondon, Helbert, additional, Sambandam, Kamalanathan K., additional, Sedor, John R., additional, Selewski, David T., additional, Sethna, Christine B., additional, Schelling, Jeffrey, additional, Sperati, John C., additional, Swiatecka-Urban, Agnes, additional, Tuttle, Katherine R., additional, Weisstuch, Joseph, additional, Vento, Suzanne, additional, Zhdanova, Olga, additional, Gillespie, Brenda, additional, Gipson, Debbie S., additional, Hill-Callahan, Peg, additional, Helmuth, Margaret, additional, Herreshoff, Emily, additional, Kretzler, Matthias, additional, Lienczewski, Chrysta, additional, Mansfield, Sarah, additional, Mariani, Laura, additional, Nast, Cynthia C., additional, Robinson, Bruce M., additional, Troost, Jonathan, additional, Wladkowski, Matthew, additional, Zee, Jarcy, additional, Zinsser, Dawn, additional, and Guay-Woodford, Lisa M., additional

Published
2020
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224. Proteomic Analysis of Complement Proteins in Membranous Nephropathy

Author

Ravindran, Aishwarya, primary, Madden, Benjamin, additional, Charlesworth, M. Cristine, additional, Sharma, Rishi, additional, Sethi, Amit, additional, Debiec, Hanna, additional, Cattran, Daniel, additional, Fervenza, Fernando C., additional, Smith, Richard J., additional, Ronco, Pierre, additional, and Sethi, Sanjeev, additional

Published
2020
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227. Dendrin in IgA nephropathy: protective or marker of early disease?

Author

Coppo, Rosanna and Fervenza, Fernando C

Subjects
*IGA glomerulonephritis, *LUPUS nephritis, *FOCAL segmental glomerulosclerosis, *TRANSFORMING growth factors
Abstract

When stratifying patients according to eGFR and Oxford classification scores, IgAN/IgAVN patients with segmental sclerosis (S1) and lower eGFR had a significantly lower dendrin gene expression. In patients with immunoglobulin A nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN) the development of proteinuria is the key clinical feature. More than a sign that "sleeping deprived podocytes" with increased dendrin expression are protected from severe disease and progression, we might suppose that I dendrin i mRNA expression is a sign of activation of a potentially dangerous process that with time will lead to podocyte apoptosis and glomerular sclerosis. [Extracted from the article]

Published
2023
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228. The association of microhematuria with mesangial hypercellularity, endocapillary hypercellularity, crescent score and renal outcomes in immunoglobulin A nephropathy

Author

Bobart, Shane A, primary, Alexander, Mariam P, additional, Shawwa, Khaled, additional, Vaughan, Lisa E, additional, Ghamrawi, Ranine, additional, Sethi, Sanjeev, additional, Cornell, Lynn, additional, Glassock, Richard J, additional, Fervenza, Fernando C, additional, and Zand, Ladan, additional

Published
2019
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229. The association of serum interleukin-6 levels with clinical outcomes in antineutrophil cytoplasmic antibody-associated vasculitis

Author

Berti, Alvise, primary, Warner, Roscoe, additional, Johnson, Kent, additional, Cornec, Divi, additional, Schroeder, Darrell R., additional, Kabat, Brian F., additional, Langford, Carol A., additional, Kallenberg, Cees G.M., additional, Seo, Philip, additional, Spiera, Robert F., additional, St Clair, E. William, additional, Fervenza, Fernando C., additional, Stone, John H., additional, Monach, Paul A., additional, Specks, Ulrich, additional, and Merkel, Peter A., additional

Published
2019
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232. Serum Biomarkers of Disease Activity in Longitudinal Assessment of Patients with ANCA‐Associated Vasculitis.

Author

Monach, Paul A., Warner, Roscoe L., Lew, Robert, Tómasson, Gunnar, Specks, Ulrich, Stone, John H., Fervenza, Fernando C., Hoffman, Gary S., Kallenberg, Cees G. M., Langford, Carol A., Seo, Philip, St. Clair, E. William, Spiera, Robert, Johnson, Kent J., and Merkel, Peter A.

Subjects
LIPOCALIN-2, PROPORTIONAL hazards models, MATRIX metalloproteinases, VASCULITIS
Abstract

Objective: Improved biomarkers of current disease activity and prediction of relapse are needed in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). For clinical relevance, biomarkers must perform well longitudinally in patients on treatment and in patients with nonsevere flares. Methods: Twenty‐two proteins were measured in 347 serum samples from 74 patients with AAV enrolled in a clinical trial. Samples were collected at Month 6 after remission induction, then every 3 months until Month 18, or at the time of flare. Associations of protein concentrations with concurrent disease activity and with future flare were analyzed using mixed‐effects models, Cox proportional hazards models, and conditional logistic regression. Results: Forty‐two patients had flares during the 12‐month follow‐up period, and 32 remained in remission. Twenty‐two patients had severe flares. Six experimental markers (CXCL13, IL‐6, IL‐8, IL‐15, IL‐18BP, and matrix metalloproteinase‐3 [MMP‐3]) and ESR were associated with disease activity using all three methods (P < 0.05, with P < 0.01 in at least one method). A rise in IL‐8, IL‐15, or IL‐18BP was associated temporally with flare. Combining C‐reactive protein (CRP), IL‐18BP, neutrophil gelatinase‐associated lipocalin (NGAL), and sIL‐2Rα improved association with active AAV. CXCL13 and MMP‐3 were increased during treatment with prednisone, independent of disease activity. Marker concentrations during remission were not predictive of future flare. Conclusion: Serum biomarkers of inflammation and tissue damage and repair have been previously shown to be strongly associated with severe active AAV were less strongly associated with active AAV in a longitudinal study that included mild flares and varying treatment. Markers rising contemporaneously with flare or with an improved association in combination merit further study. [ABSTRACT FROM AUTHOR]

Published
2022
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235. Preferential Binding of Anti-Neutrophil Cytoplasmic Antibodies to an Unexpected Epitope of a Chimeric Proteinase 3 Mutant

Author

Moura, Marta Casal, Thompson, Gwen E., Nelson, Darlene A., Fussner, Lynn A., Hummel, Amber M., Jenne, Dieter E., Emerling, Daniel, Volkmuth, Wayne, Fervenza, Fernando C., Kallenberg, Cees G.M., Langford, Carol A., McCune, Joseph W., Merkel, Peter A., Monach, Paul A., Seo, Philip, Spiera, Robert F., St. Clair, E. William, Ytterberg, Steven R., Stone, John H., Robinson, William H., Pang, Yuan-Ping, and Specks, Ulrich

Subjects
cardiovascular diseases
Abstract

Proteinase 3 (PR3) is the major antigen for anti-neutrophil cytoplasmic antibodies (ANCAs) in the systemic autoimmune vasculitis, granulomatosis with polyangiitis (GPA). PR3-targeting ANCAs (PR3-ANCAs) recognize different epitopes on PR3 and are thought to be pathogenic for the development of the necrotizing vasculitis. To identify epitopes recognized by PR3-ANCAs, we pursued a strategy based on human-murine chimeric PR3 mutants. Interestingly, rather than observing reduced binding of PR3-ANCAs to Epitope 5 on a PR3 mutant (iHm5-Val 103 ) with chimeric mutations in Epitope 5, we found substantially increased binding of the majority of PR3-ANCAs to iHm5-Val 103 compared with the PR3 mutant (iPR3-Val 103 ) clinically used to detect PR3-ANCAs. More interestingly, using iHm5-Val 103 we identified a monoclonal antibody (moANCA518) from a patient with GPA that bound selectively to iHm5-Val 103 . Inhibition experiments using epitope-specific monoclonal antibodies and their antigen-binding fragments mapped the binding sites of moANCA518 and PR3-ANCAs (from patients displaying preferential binding to iHm5-Val 103 over iPR3-Val 103 ) to Epitope 3 on iHm5-Val 103 , a mutation-free epitope located far from the mutation sites in Epitope 5. These results demonstrate that the selective binding of moANCA518 (and likely the preferential binding of PR3-ANCAs from patients) to iHm5-Val 103 is conferred by increased antigenicity of Epitope 3 on iHm5-Val 103 caused by distal mutations, indicating that PR3-ANCAs bind to epitopes of a folded antigen conducive to allosteric effects of mutations—a previously unrecognized characteristic with implications for studying antibody-mediated autoimmune diseases and novel treatment approaches.

Published
2019
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241. Comparison of treatment options in adults with frequently relapsing or steroid-dependent minimal change disease.

Author

Heybeli, Cihan, Erickson, Stephen B, Fervenza, Fernando C, Hogan, Marie C, Zand, Ladan, and Leung, Nelson

Subjects
CALCINEURIN, CYCLOPHOSPHAMIDE, MYCOPHENOLIC acid, RITUXIMAB, DISEASE progression
Abstract

Background Studies comparing all treatment options for frequently-relapsing/steroid-dependent (FR/SD) minimal change disease (MCD) in adults are lacking. Methods Medical records of 76 adults with FR/SD MCD who were treated with corticosteroids as the first-line therapy were reviewed. Treatment options were compared for the time to relapse, change of therapy and progression (relapse on full-dose treatment). Results Second-line treatments included rituximab (RTX; n  = 13), mycophenolate mofetil (MMF; n  = 12), calcineurin inhibitors (CNI; n  = 26) and cyclophosphamide (CTX; n  = 16). During the second-line treatments, 48 (71.6%) patients relapsed at median 17 (range 2–100)  months. The majority of relapses occurred during dose tapering or off drug. Twenty of 65 (30.8%) changed therapy after the first relapse. The median time to relapse after the second line was 66 versus 28 months in RTX versus non-RTX groups (P = 0.170). The median time to change of treatment was 66 and 44 months, respectively (P = 0.060). Last-line treatment options included RTX (n  = 8), MMF (n  = 4), CNI (n  = 3) and CTX (n  = 2). Seven (41.2%) patients had a relapse during the last-line treatment at median 39 (range 5–112)  months. The median time to relapse was 48 versus 34 months in the RTX versus non-RTX groups (P = 0.727). One patient in the RTX group died presumably of heart failure. No major adverse event was observed. During the median follow-up of 81 (range 9–355)  months, no patients developed end-stage renal disease. Conclusions Relapse is frequent in MCD in adults. Patients treated with RTX may be less likely to require a change of therapy and more likely to come off immunosuppressive drugs. [ABSTRACT FROM AUTHOR]

Published
2021
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246. Hematopoietic Stem Cell Transplant-Membranous Nephropathy Is Associated with Protocadherin FAT1

Author

Sethi, Sanjeev, Madden, Benjamin, Casal Moura, Marta, Nasr, Samih H., Klomjit, Nattawat, Gross, LouAnn, Negron, Vivian, Charlesworth, M. Cristine, Alexander, Mariam P., Leung, Nelson, Specks, Ulrich, Fervenza, Fernando C., and Haas, Mark

Abstract

Hematopoietic stem cell transplant (HSCT) is a treatment for certain hematologic malignancies and immune disorders. A complication of HSCT is membranous nephropathy (MN), which results from antibodies targeting an antigen in the glomerular basement membrane (GBM). The antigen in most cases of HSCT-associated MN is not known. Laser microdissection and mass spectrometry identified a novel protein, protocadherin FAT1 (FAT1), in HSCT-associated MN. Kidney biopsy specimens showed granular staining for FAT1 along the GBM in HSCT-associated MN. Antibodies to FAT1 were detected in serum and in kidney biopsy tissue in HSCT-associated MN, but not in controls. FAT1-associated MN appears to be a unique type of MN associated with HSCT. FAT1-associated MN represents the majority of MN associated with HSCT.

Published
2022
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247. Acute glomerulonephritis

Author

Sethi, Sanjeev, De Vriese, An S, and Fervenza, Fernando C

Abstract

Glomerulonephritis is a heterogeneous group of disorders that present with a combination of haematuria, proteinuria, hypertension, and reduction in kidney function to a variable degree. Acute presentation with full blown nephritic syndrome or rapidly progressive glomerulonephritis is uncommon and is mainly restricted to patients with post-infectious glomerulonephritis, anti-neutrophil cytoplasmic antibodies-associated vasculitis, and anti-glomerular basement membrane disease. Most frequently, patients present with asymptomatic haematuria and proteinuria with or without reduced kidney function. All glomerulonephritis disorders can show periods of exacerbation, but disease flairs characteristically occur in patients with IgA nephropathy or C3 glomerulopathy. The gold standard for the diagnosis of a glomerulonephritis is a kidney biopsy, with a hallmark glomerular inflammation that translates into various histopathological patterns depending on the location and severity of the glomerular injury. Traditionally, glomerulonephritis was classified on the basis of the different histopathological patterns of injury. In the last few years, substantial progress has been made in unravelling the underlying causes and pathogenetic mechanisms of glomerulonephritis and a causal approach to the classification of glomerulonephritis is now favoured over a pattern-based approach. As such, glomerulonephritis can be broadly classified as immune-complex glomerulonephritis (including infection-related glomerulonephritis, IgA nephropathy, lupus nephritis, and cryoglobulinaemic glomerulonephritis), anti-neutrophil cytoplasmic antibodies-associated (pauci-immune) glomerulonephritis, anti-glomerular basement membrane glomerulonephritis, C3 glomerulopathy, and monoclonal immunoglobulin-associated glomerulonephritis. We provide an overview of the clinical presentation, pathology, and the current therapeutic approach of the main representative disorders in the spectrum of glomerulonephritis.

Published
2022
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