SFB 1192
Project C1
Hamburg Glomerulonephritis Registry
Immune-mediated glomerular diseases represent one of the main causes for end-stage kidney disease. Because of the significant morbidity and mortality caused by these diseases, better diagnosis and treatment options are urgently needed. Albeit important discoveries have been made in the last years in the field, specific biomarkers and treatment options for immune-mediated glomerular diseases are still largely missing. The aim of the “Hamburg Glomerulonephritis Registry” is to enable the transfer of discoveries on pathomechanistic insights of glomerulonephritis into clinical care. For this, not only large, well characterized clinical cohorts with integrated human tissue biobanking are needed, but also the integration and implementation of novel experimental tools and technologies into relevant clinical settings. After successfully implementing the “Hamburg Glomerulonephritis Registry” as a central part of the SFB1192, we already exceeded our recruitment goal of 1500 patients with glomerulonephritis. In the next funding period, we will therefore focus on the rigorous prospective follow-up of these patients, including biomaterials (kidney biopsy specimen, serum, DNA, PBMC etc.) and clinical data. The “Hamburg Glomerulonephritis Registry” will provide a unique platform for all projects of this consortium to enable: 1) The characterization of specific diagnostic and therapeutic biomarkers and pathogenetic mechanisms in patients with glomerulonephritis; 2) The development and clinical implementation of automated histo-morphologic and multiplex-based classification and scoring algorithms; 3) Clinical transfer of pathogenetic mechanisms derived from animal experiments and in vitro studies; 4) Validation of molecular disease signatures in patients with glomerulonephritis; 5) Provide a platform to facilitate genetic studies, recruitment of patients for clinical trials and international cooperation with academic and industrial partners. The aim of the Hamburg Glomerulonephritis Registry is to facilitate the development of novel pathogenesis-based, individualized diagnostic biomarkers and therapeutic strategies for immune-mediated glomerular diseases.
Publications with support of C1
Diagnostic role of renal biopsy in PLA2R1-antibody positive patients with nephrotic syndrome.
Wiech T, Stahl RAK, Hoxha E (2019).
Mod Pathol. In Print.
Genetics of membranous nephropathy.
Gupta S, Köttgen A, Hoxha E, Brenchley P, Bockenhauer D, Stanescu HC, Kleta R (2018).
Nephrol Dial Transplant 33:1493-1502
Antigen-Specific IgG Subclasses in Primary and Malignancy-Associated Membranous Nephropathy.
von Haxthausen F, Reinhard L, Pinnschmidt HO, Rink M, Soave A, Hoxha E*, Stahl RAK* (2018).
Front Immunol 9:3035. doi: 10.3389/fimmu.2018.03035. . *: equal contribution.
Bevacizumab-associated glomerular microangiopathy.
Person F, Rinschen MM, Brix SR, Wulf S, Noriega MLM, Fehrle W, Schmitz J, Schwarz A, Ivanyi P, Steinmetz OM, Reinhard L, Hoxha E, Zipfel PF, Bräsen JH, Wiech T (2018).
Mod Pathol doi: 10.1038/s41379-018-0186-4. [Epub ahead of print]
Organisation of lymphocytic infiltrates in ANCA-associated glomerulonephritis.
Brix SR, Noriega M, Herden EM, Goldmann B, Langbehn U, Busch M, Jabs WJ, Steinmetz OM, Panzer U, Huber TB, Stahl RAK, Wiech T (2018).
Histopathology 72:1093-1101.
IL-17C/IL-17 Receptor E Signaling in CD4+ T Cells Promotes TH17 Cell-Driven Glomerular Inflammation.
Krohn S, Nies JF, Kapffer S, Schmidt T, Riedel JH, Kaffke A, Peters A, Borchers A, Steinmetz OM, Krebs CF, Turner JE, Brix SR, Paust HJ, Stahl RAK, Panzer U (2018).
J Am Soc Nephrol 29:1210-1222.
Development and validation of a renal risk score in ANCA-associated glomerulonephritis.
Brix SR, Noriega M, Tennstedt P, Vettorazzi E, Busch M, Nitschke M, Jabs WJ, Özcan F, Wendt R, Hausberg M, Sellin L, Panzer U, Huber TB, Waldherr R, Hopfer H, Stahl RAK, Wiech T (2018).
Kidney Int 94:1177-1188.
The Most N-Terminal Region of THSD7A Is the Predominant Target for Autoimmunity in THSD7A-Associated Membranous Nephropathy.
Seifert L*, Hoxha E*, Eichhoff AM, Zahner G, Dehde S, Reinhard L, Koch-Nolte F, Stahl RAK, Tomas NM (2018).
J Am Soc Nephrol 29:1536-1548. *: equal contribution.
Immunohistochemical and serological characterization of membranous nephropathy in children and adolescents.
Dettmar AK, Wiech T, Kemper MJ, Soave A, Rink M, Oh J, Stahl RAK, Hoxha E (2018).
Pediatr Nephrol 33:463-472
A Heterologous Model of Thrombospondin Type 1 Domain-Containing 7A-Associated Membranous Nephropathy.
Tomas NM, Meyer-Schwesinger C, von Spiegel H, Kotb AM, Zahner G, Hoxha E, Helmchen U, Endlich N, Koch-Nolte F, Stahl RAK (2017).
J Am Soc Nephrol 28:3262-3277
THSD7A expression in human cancer.
Stahl PR, Hoxha E, Wiech T, Schröder C, Simon R, Stahl RA (2017).
Genes Chromosomes Cancer 56:314-327
An Indirect Immunofluorescence Method Facilitates Detection of Thrombospondin Type 1 Domain-Containing 7A-Specific Antibodies in Membranous Nephropathy.
Hoxha E, Beck LH Jr, Wiech T, Tomas NM, Probst C, Mindorf S, Meyer-Schwesinger C, Zahner G, Stahl PR, Schöpper R, Panzer U, Harendza S, Helmchen U, Salant DJ, Stahl RA (2017).
J Am Soc Nephrol 28:520-531
Autoimmune Renal Disease Is Exacerbated by S1P-Receptor-1-Dependent Intestinal Th17 Cell Migration to the Kidney.
Krebs CF, Paust HJ, Krohn S, Koyro T, Brix SR, Riedel JH, Bartsch P, Wiech T, Meyer-Schwesinger C, Huang J, Fischer N, Busch P, Mittrücker HW, Steinhoff U, Stockinger B, Perez LG, Wenzel UO, Janneck M, Steinmetz OM, Gagliani N, Stahl RAK, Huber S, Turner JE, Panzer U (2016).
Immunity 45:1078-1092.
Autoantibodies against thrombospondin type 1 domain-containing 7A induce membranous nephropathy.
Tomas NM*, Hoxha E*, Reinicke AT, Fester L, Helmchen U, Gerth J, Bachmann F, Budde K, Koch-Nolte F, Zahner G, Rune G, Lambeau G, Meyer-Schwesinger C§, Stahl RA§ (2016).
J Clin Invest 126:2519-2532. *§: equal contribution.
A Mechanism for Cancer-Associated Membranous Nephropathy.
Hoxha E, Wiech T, Stahl PR, Zahner G, Tomas NM, Meyer-Schwesinger C, Wenzel U, Janneck M, Steinmetz OM, Panzer U, Harendza S, Stahl RA (2016).
N Engl J Med 374:1995-1996
Project-Team
Project Leaders
PD Dr. Maja Lindenmeyer
Prof. Dr. Thorsten Wiech
Prof. Dr. Tobias B. Huber
Co-Workers
Katharina Schulz
Sonia Wulf
Eva-Maria Kuhr