What are the guideline sources, guidelines and references for Anti-glomerular basement membrane disease ?
Guideline sources The following summarized guidelines for the evaluation and management of anti-GBM glomerulonephritis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), the American Society for Apheresis (ASFA 2019), and the Japanese Society of Nephrology (JSN 2016). 1 2 3 4 5 5 6 Definition Anti-GBM disease, previously called Goodpasture's disease, is a systemic autoimmune disorder resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. 4 Epidemiology Anti-GBM disease is caused by an autoimmune response against type IV collagen molecules in the kidneys and lungs triggered by environmental factors in genetically predisposed individuals. 5 Disease course https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 1/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway Autoantibodies against type IV collagen molecules in the kidneys and lungs result in anti-GBM disease, which causes clinical manifestations of RPGN with concurrent lung hemorrhage. Disease progression may lead to dialysis-dependent kidney failure, end-stage renal disease necessitating kidney transplantation, and respiratory failure. 5 Prognosis and risk of recurrence Anti-GBM disease is associated with an in-hospital mortality rate of 7.7 per 100 admissions. 6 Guidelines 1. Diagnostic investigations Initial evaluation: obtain prompt evaluation for anti-GBM disease in all patients with suspected RPGN. B Show 3 more 2. Diagnostic procedures Kidney biopsy: As per KDIGO 2021 guidelines, perform kidney biopsy with immunofluorescence at presentation or within 24 hours in patients with suspected RPGN. B As per JSN 2016 guidelines, perform kidney biopsy to aid in determining the treatment strategy for RPGN. B 3. Medical management General principles: Initiate treatment without delay in patients with suspected anti-GBM disease, even before the diagnosis is confirmed. B Consider offering a conservative approach in patients without alveolar hemorrhage but with oliguria and/or advanced kidney failure requiring dialysis, especially in the presence of a very high proportion of crescents (85-100%) on kidney biopsy. C Immunosuppressive therapy: As per KDIGO 2021 guidelines: Initiate immunosuppression with cyclophosphamide and corticosteroid plus plasmapheresis in all patients with anti-GBM glomerulonephritis, except patients receiving dialysis at presentation, having 100% crescents or > 50% global glomerulosclerosis in an adequate biopsy sample, and not having a pulmonary hemorrhage. B Administer cyclophosphamide for 2-3 months and corticosteroids for about 6 months. B https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 2/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway As per JSN 2016 guidelines, initiate high-dose corticosteroids with immunosuppressive agents as initial therapy in patients with anti-GBM disease presenting with RPGN. Initiate corticosteroids alone, in combination with plasmapheresis, only if the use of immunosuppressive agents is not desirable. B Show 5 more Intravenous immunoglobulin: consider initiating IVIG in patients with concurrent complications such as severe infections to avoid the standard therapy with high-dose corticosteroids and immunosuppressants. C Maintenance therapy: As per KDIGO 2021 guidelines: Do not initiate maintenance therapy for anti-GBM. D Initiate maintenance therapy in anti-GBM- and ANCA-positive patients as for patients with ANCA-associated vasculitis. B As per JSN 2016 guidelines, consider initiating low-dose corticosteroids as maintenance therapy to improve renal function and survival in patients with anti-GBM disease presenting with RPGN. C Show 2 more Antithrombotic therapy: consider initiating anticoagulant or antiplatelet therapy in the absence of hemorrhagic lesions. C 4. Therapeutic procedures Plasmapheresis: As per KDIGO 2021 guidelines: Initiate plasmapheresis along with immunosuppressive therapy in all patients with anti-GBM glomerulonephritis, except patients receiving dialysis at presentation, having 100% crescents or > 50% global glomerulosclerosis in an adequate biopsy sample, and not having a pulmonary hemorrhage. B Perform plasmapheresis until anti-GBM titers are no longer detectable. B As per JSN 2016 guidelines, initiate plasmapheresis in addition to immunosuppressive therapy for improved renal function and survival in patients with anti-GBM antibody-positive RPGN. B 5. Surgical interventions Kidney transplantation: postpone kidney transplantation in patients with kidney failure due to anti-GBM disease until anti-GBM antibodies remain undetectable for 6 months. B 6. Preventative measures https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 3/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway Prophylaxis for Pneumocystis jirovecii pneumonia: initiate TMP-sulfamethoxazole to improve life prognosis in patients with RPGN receiving with immunosuppressive therapy. A 7. Follow-up and surveillance Assessment of treatment response: As per KDIGO 2021 guidelines, monitor kidney function, pulmonary infiltrates, anti-GBM antibody titers, and blood counts. Modify treatment accordingly. B As per JSN 2016 guidelines, consider measuring anti-GBM antibody titers to monitor the disease activity and recurrence. Management of refractory disease: consider initiating rituximab in patients with refractory anti- GBM disease. C Likelihood Ratios Pertinent positives The following findings increase the probability of anti-GBM glomerulonephritis in adults. -1 Finding LR+ Value Positive anti-GBM antibodies 2.82 Pertinent negatives The following findings decrease the probability of anti-GBM glomerulonephritis in adults. -1 Finding LR- Value Negative anti-GBM antibodies 0.69 References 1. Kidney Disease: Improving Global Outcomes KDIGO Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100 4S S1 S276. Open 2. Yoshihiro Arimura, Eri Muso, Shoichi Fujimoto et al. Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. Clin Exp Nephrol. 2016 Jun;20 3 322 41. Open 3. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 4. Andrew L Chan, Samuel Louie, Kevin O Leslie et al. Cutting edge issues in Goodpasture's disease. 2011 Oct;41 2 151 62.2011 Oct;41 2 151 62. Open https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 4/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway 5. Stephen P McAdoo, Charles D Pusey. Anti-Glomerular Basement Membrane Disease. 2017 Jul 7;12 7 1162 1172.2017 Jul 7;12 7 1162 1172. Open 6. Wisit Kaewput, Charat Thongprayoon, Boonphiphop Boonpheng et al. Inpatient Burden and Mortality of Goodpasture's Syndrome in the United States: Nationwide Inpatient Sample 2003 2014. 2020 Feb 6;9 2 455.2020 Feb 6;9 2 455. Open 7. KDIGO. Chapter 14 Anti-glomerular basement membrane antibody glomerulonephritis. Kidney Int Suppl 2011 . 2012 Jun;2 2 240 242. 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