Atthaphong Phongphithakchai
1* 
, Suntornwit Praditau-Krit
1 , Phongsak Dandecha
1 , Ussanee Boonsrirat
1 , Poowadon Wetwittayakhlung
2 1 Division of Nephrology, Department of medicine, Faculty of Medicine, Prince of Songkla University, Songkhla, Thailand
2 Department of Pathology, Faculty of Medicine, Prince of Songkla University, Songkhla, Thailand
Abstract
Anti-glomerular basement membrane (anti-GBM) nephritis is uncommon glomerular disease caused by autoantibodies targeting the capillary beds of the kidney. The clinical presentation of the disease is a variable nephritic syndrome, rapidly progressing to glomerulonephritis. Treatment outcomes are dependent on predictors at first diagnosis. We presented a case of 58-year-old man who did not have underlying disease presented with marked abdominal distension and acute kidney injury. He had no evidence of chronic renal disease before admission however, laboratory test showed microscopic haematuria (RBC 30-50 per high-powered field), proteinuria (2.9 g/d), and renal failure (serum creatinine 610 µmol/L) compatible with rapidly progressive glomerulonephritis; hence, a renal biopsy was conducted. The pathology showed 100% crescentic glomerulonephritis with IgG deposits in a linear pattern at the GBM. The initial serum anti-GBM titre was 105.59 RU/mL. This patient had poor renal prognosis factors for treatment response. After a discussion regarding treatment option with the patient, we decided to give intensive immunosuppressive therapy and plasmapheresis due to his good baseline functional status. The patient achieved partial remission and is not dialysis dependent. In conclusion, despite a poor renal prognosis with 100% crescents and serum creatinine ≥ 600 µmol/L, the treated patient had a good survival status and did not become dialysis-dependent. However, immunosuppressive treatment should be performed along with careful monitoring for infection to avoid infection-related morbidity and mortality.
Implication for health policy/practice/research/medical education:
Anti-glomerular basement membrane nephritis is a rare glomerular disease characterized by IgG deposit at the glomerular basement membrane and crescentic lesion. The 100% crescentic lesion is related to unfavorable prognosis and is challenging to treat with the immunosuppressive drug. Here we reported the successful treatment with the intense immunosuppressive therapy in a 58-year-old male with 100%crescentic lesion of anti-GBM nephritis preventing to become dialysis-dependent status.
Please cite this paper as: Phongphithakchai A, Praditau-Krit S, Dandecha P, Boonsrirat U, Wetwittayakhlung P. Successful treatment with intense immunosuppressive therapy in an initially 100% crescentic lesion of anti-GBM nephritis. J Nephropathol. 2022;11(x):exx. DOI: 10.34172/jnp.2022.xx.