Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients

We report the overall and renal outcome in a French nationwide multicenter cohort of 119 patients with anti-glomerular basement membrane (anti-GBM) disease. Sixty-four patients (54%) had an exclusive renal involvement, 7 (6%) an isolated alveolar hemorrhage and 48 (40%) a combined renal and pulmonary involvement. Initial renal replacement therapy (RRT) was required in 78% of patients; 82% received plasmapheresis, 82% cyclophosphamide, and 9% rituximab. ANCA positive (28%) patients were older (70 vs. 47 years, p < 0.0001), less frequently smokers (26 vs. 54%, p = 0.03), and had less pulmonary involvement than ANCA- patients. The 5 years overall survival was 92%. Risk factors of death (n = 11, 9.2%) were age at onset [HR 4.10 per decade (1.89–8.88) p = 0.003], hypertension [HR 19.9 (2.52–157 0.2) p = 0.005], dyslipidemia [HR 11.1 (2.72–45) p = 0.0008], and need for mechanical ventilation [HR 5.20 (1.02–26.4) p = 0.047]. The use of plasmapheresis was associated with better survival [HR 0.29 (0.08–0.98) p = 0.046]. At 3 months, 55 (46%) patients had end-stage renal disease (ESRD) vs. 37 (31%) ESRD-free and 27 (23%) unevaluable with follow-up < 3 months. ESRD patients were older, more frequently female and had a higher serum creatinine level at presentation than those without ESRD. ESRD-free survival was evaluated in patients alive without ESRD at 3 months (n = 37) using a landmark approach. In conclusion, this large French nationwide study identifies prognosis factors of renal and overall survival in anti-GBM patients.

[1]  D. Metze,et al.  Nomenclature of cutaneous vasculitides – German translation of the dermatologic addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides , 2018, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG.

[2]  M. Segelmark,et al.  Anti-glomerular basement membrane disease: an update on subgroups, pathogenesis and therapies. , 2018, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[3]  C. Zeng,et al.  Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease , 2018, Journal of Clinical Pathology.

[4]  G. Heinze,et al.  An unjustified benefit: immortal time bias in the analysis of time‐dependent events , 2018, Transplant international : official journal of the European Society for Organ Transplantation.

[5]  R. Wolterbeek,et al.  Predicting Outcome in Patients with Anti-GBM Glomerulonephritis. , 2018, Clinical journal of the American Society of Nephrology : CJASN.

[6]  V. Tesar,et al.  Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients , 2017, Kidney international.

[7]  C. Pusey,et al.  Anti-Glomerular Basement Membrane Disease. , 2017, Clinical journal of the American Society of Nephrology : CJASN.

[8]  D. Chauveau,et al.  Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis. , 2016, Journal of autoimmunity.

[9]  Cathal Walsh,et al.  Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease. , 2016, Clinical journal of the American Society of Nephrology : CJASN.

[10]  N. Anguel,et al.  Rituximab in anti-GBM disease: A retrospective study of 8 patients. , 2015, Journal of autoimmunity.

[11]  K. Farrington,et al.  Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort. , 2015, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[12]  M. de Vincentiis,et al.  Goodpasture's syndrome: a clinical update. , 2015, Autoimmunity reviews.

[13]  R. McGill,et al.  Anti-glomerular basement membrane disease treated with mycophenolate mofetil, corticosteroids, and plasmapheresis. , 2013, Clinical nephrology.

[14]  David W. Johnson,et al.  Anti-glomerular basement membrane antibody disease is an uncommon cause of end-stage renal disease. , 2013, Kidney international.

[15]  J. D. de Zoysa,et al.  Incidence and features of dual anti‐GBM‐positive and ANCA‐positive patients , 2011, Nephrology.

[16]  K. Abbott,et al.  Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease. , 2011, Journal of the American Society of Nephrology : JASN.

[17]  Ming-hui Zhao,et al.  Anti-Glomerular Basement Membrane Disease: Outcomes of Different Therapeutic Regimens in a Large Single-Center Chinese Cohort Study , 2011, Medicine.

[18]  P. van Paassen,et al.  Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. , 2005, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[19]  T. Dougan,et al.  Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. , 2004, Kidney international.

[20]  C. Pusey.  Anti-glomerular basement membrane disease. , 2003, Kidney international.

[21]  Sunil J Rao,et al.  Regression Modeling Strategies: With Applications to Linear Models, Logistic Regression, and Survival Analysis , 2003 .

[22]  J. Levy,et al.  Long-Term Outcome of AntiGlomerular Basement Membrane Antibody Disease Treated with Plasma Exchange and Immunosuppression , 2001, Annals of Internal Medicine.

[23]  C. García-Cantón,et al.  Goodpasture's syndrome treated with mycophenolate mofetil. , 2000, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[24]  A. Rees,et al.  Anti-Glomerular Basement Membrane Disease , 2020, Definitions.

[25]  M. Daha,et al.  Sequential development of anti-GBM nephritis and ANCA-associated Pauci-immune glomerulonephritis. , 1999, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[26]  A. Collins,et al.  Comparison of anti-GBM antibodies in sera with or without ANCA. , 1997, Journal of the American Society of Nephrology : JASN.

[27]  W. Breitbart,et al.  Clinical update , 1994, Psycho-oncology.

[28]  L. Revert,et al.  Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease. , 1991, Clinical nephrology.

[29]  D. Jayne,et al.  Autoantibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis. , 1990, Kidney international.

[30]  J. Greene,et al.  Goodpasture's syndrome: A report of five cases and review of the literature , 1970 .

[31]  P. Doolan,et al.  FINDINGS SUGGESTIVE OF A PULMONARY ALVEOLAR-CAPILLARY BLOCK SYNDROME IN A PATIENT WITH CHRONIC LYMPHOCYTIC LEUKEMIA. , 1964, The American journal of medicine.

[32]  C. Krakower,et al.  Localization of the nephrotoxic antigen within the isolated renal glomerulus. , 1951, A.M.A. archives of pathology.

[33]  J. Hardy,et al.  A Clinicopathologic Entity , 2017 .

[34]  P. Merkel,et al.  2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. , 2013, Arthritis and rheumatism.

[35]  A. Kıykım,et al.  Successful treatment of resistant antiglomerular basement membrane antibody positivity with mycophenolic acid. , 2010, Internal medicine.

[36]  E. Ritz,et al.  Rapidly progressive glomerulonephritis: analysis of prevalence and clinical course. , 1991, Nephron.