Hesitating on Receiving Treatment for Rapidly Proliferative Glomerulonephritis

Introduction: ANCA vasculitis, is an inflammatory condition affecting small arteries and causing tissue damage in various organs. Renal involvement can lead to ESRD with a high mortality rate. Prevalence ranges from 4.6 to 21.8 cases per 1 million person-years globally. RPGN is a severe renal manifestation, such as nephrotic proteinuria, hypertension, and azotemia. Diagnosis involves serological testing for ANCAs and kidney biopsy. This is a case of RPGN with ANCA(+) in a patient hesitant to receive therapy. Case Description: A 73 y/o female with PMHx of active smoking, breast cancer, and HTN who was referred to nephrology clinic due to elevated serum creatinine, hematuria, and foamy urine for 4 months. Vital signs with 152/94 mmHg, 78 bpm, and 96% oxygen. Laboratory revealed UPCR of 4.6 grams/day and a creatinine of 2.12 mg/dL from 0.88. Biomarkers showed an ANA titer 1:2560, RF 22, p-ANCA (+), ESR 95; CRP 31.7. Started Solumedrol 500 mg IV every 12 hours for 3 days and a kidney biopsy was done. Differential diagnoses of ANCA vasculitis, lupus nephritis, and C3GN considered. Workup showed MPO (+), C3 138, C4 12, anti-dsDNA(-), anti-Smith (-), and anti-GBM (-). Despite steroid induction, renal function and HTN worsened. Rituximab initiated and she developed sudden headache and blurry vision. MRI revealed an acute ischemic left occipital stroke and an old lacunar infarct. Final biopsy showed immune complex-mediated necrotizing glomerulonephritis with epithelial crescents and interstitial fibrosis. Despite advice to continue therapy, she refused due to perceived association with her acute stroke. Also, denied cyclophosphamide and agreed to receive azathioprine and prednisone. On follow-up, she continued to have severe proteinuria and de novo CHF due to poor compliance with regimen. Discussion: ANCA glomerulonephritis requires induction and maintenance therapy. Severe cases may require treatment such as methylprednisolone, cyclophosphamide, and plasmapheresis with prednisone. Maintenance therapy, rituximab or azathioprine with prednisone, this is crucial for preventing relapse. Patient involvement in treatment and education is essential for achieving goals and ensuring compliance. Despite advancements, relapse rates remain significant. Close monitoring for treatment resistance and lack of remission enables healthcare providers to optimize outcomes for ANCA GN patients.