Elion Hoxha1, Sigrid Harendza1, Hans O Pinnschmidt2, Nicola M Tomas1, Udo Helmchen3, Ulf Panzer1, Rolf A K Stahl1. 1. III. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. 2. Institut für Medizinische Biometrie & Epidemiologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. 3. Nierenregister, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany.
Abstract
BACKGROUND: Phospholipase A2 receptor antibodies (PLA2R-Ab) and thrombospondin type-1 domain-containing 7A antibodies (THSD7A-Ab) are present in 70-80% of patients with membranous nephropathy (MN). Little, however, is known about the pathogenesis of MN and the clinical outcome in PLA2R-Ab- and THSD7A-Ab-negative patients. METHODS: In this prospective multicentre observational study, the clinical outcome of 37 patients with biopsy-proven MN who were negative for PLA2R-Ab and THSD7A-Ab in the serum was analysed. RESULTS: A total of 198 patients were screened for inclusion in the study. Of these, 157 patients were positive for PLA2R-Ab and 4 patients for THSD7A-Ab. The remaining 37 patients were negative for both antibodies were and included in this study. Six patients died during the follow-up, five because of malignant diseases and one of an infection. One patient went into end-stage renal disease, and two patients were lost to follow-up. The remaining 28 patients were followed for at least 24 months (35.6 ± 8.9 months). Seventeen patients received immunosuppressive (IS) therapy, and 11 received supportive care only. At the end of the follow-up, 14 of the 17 patients treated with immunosuppressants and 10 of 11 patients on supportive therapy had a remission of proteinuria. The time to reach remission of proteinuria and serum creatinine levels at the end of the follow-up were not different between both groups. A univariate Cox regression analysis indicated that the use of immunosuppression did not alter the chance to reach a remission of proteinuria. CONCLUSIONS: A high number of PLA2R-Ab- and THSD7A-Ab-negative patients with MN have a good prognosis and might not need IS therapy.
BACKGROUND: Phospholipase A2 receptor antibodies (PLA2R-Ab) and thrombospondin type-1 domain-containing 7A antibodies (THSD7A-Ab) are present in 70-80% of patients with membranous nephropathy (MN). Little, however, is known about the pathogenesis of MN and the clinical outcome in PLA2R-Ab- and THSD7A-Ab-negative patients. METHODS: In this prospective multicentre observational study, the clinical outcome of 37 patients with biopsy-proven MN who were negative for PLA2R-Ab and THSD7A-Ab in the serum was analysed. RESULTS: A total of 198 patients were screened for inclusion in the study. Of these, 157 patients were positive for PLA2R-Ab and 4 patients for THSD7A-Ab. The remaining 37 patients were negative for both antibodies were and included in this study. Six patients died during the follow-up, five because of malignant diseases and one of an infection. One patient went into end-stage renal disease, and two patients were lost to follow-up. The remaining 28 patients were followed for at least 24 months (35.6 ± 8.9 months). Seventeen patients received immunosuppressive (IS) therapy, and 11 received supportive care only. At the end of the follow-up, 14 of the 17 patients treated with immunosuppressants and 10 of 11 patients on supportive therapy had a remission of proteinuria. The time to reach remission of proteinuria and serum creatinine levels at the end of the follow-up were not different between both groups. A univariate Cox regression analysis indicated that the use of immunosuppression did not alter the chance to reach a remission of proteinuria. CONCLUSIONS: A high number of PLA2R-Ab- and THSD7A-Ab-negative patients with MN have a good prognosis and might not need IS therapy.
Authors: Nicola M Tomas; Catherine Meyer-Schwesinger; Hanning von Spiegel; Ahmed M Kotb; Gunther Zahner; Elion Hoxha; Udo Helmchen; Nicole Endlich; Friedrich Koch-Nolte; Rolf A K Stahl Journal: J Am Soc Nephrol Date: 2017-08-16 Impact factor: 10.121
Authors: Andrea Angioi; Nicola Lepori; Ana Coloma López; Sanjeev Sethi; Fernando C Fervenza; Antonello Pani Journal: J Nephrol Date: 2017-09-05 Impact factor: 3.902
Authors: An S De Vriese; Richard J Glassock; Karl A Nath; Sanjeev Sethi; Fernando C Fervenza Journal: J Am Soc Nephrol Date: 2016-10-24 Impact factor: 10.121