A Young Woman with Sick Kidney in Biopsy

Introduction: Rapidly progressive glomerulonephritis is characterized by a rapid decline in renal function over days to weeks with U/A with proteinuria, hematuria, dysmorphic RBC's,and RBC casts. Incidence in the United States is 7 cases per 1 million persons,while in the United Kingdom it is 2 cases per 1 million. Common etiologies include immune complex-mediated RPGN, such as lupus, IgA vasculitis, or C3 glomerulonephritis. Here we present a difficult to treat case of a young woman with rapidly progressive glomerulonephritis. Case Description: This is a case of a 27-y/o female without medical history, who presented to the emergency room with nausea, vomiting, and diarrhea over two days. Vital signs revealed high blood pressure, tachycardia, hgb 6 g/dL. Labs revealed serum creatinine: 17, BUN: 116, Na:126, K: 5.5, CO2: 13. U/A with protein >500 mg/dL, large blood, microhematuria and urine protein/creatinine 6.6 g/day. After, patient developed pleuritic chest pain, with pericardial effusion and pericardial friction rub. Emergent hemodialysis was started with improvement after 4 days of HD and pulse steroids. Other labs revealed (+) Coombs test, high ferritin and LDH levels, (+) ANA/Ro pattern, low C3, (-) C4, (-) scleroderma AB, (-) anti-RNP AB, (-) anti-Sm AB, (+) anti-Ro AB, (-) anti-dsDNA AB, and (+) lupus anticoagulant. Kidney biopsy confirmed Diffuse Proliferative Lupus Nephritis Class IV. Treatment begun with Hydroxychloroquine, Mycophenolate and high dose steroids. After treatment patient remained dependent on HD. Discussion: Kidney biopsy is required for LN diagnosis, prognosis, and adequate management. Prognosis of disease worsens with advancing WHO histopathology classes, particularly in LN Class 4, where early initiation of therapy significantly improves disease outcomes. Treatment involves pulse steroids with methylprednisolone for 1-3 days followed by oral prednisone along with either cyclophosphamide (500 mg IV every two weeks for 3 months) or mycophenolate mofetil (2-3 g/day for 6 months). Monitoring disease includes assessments of blood pressure, renal function, proteinuria, urinary sediment, C3/C4 levels, and anti-dsDNA AB measured at least biannually. Earlier disease detection is vital as achieving complete kidney response during treatment provides a 5-year patient and kidney survival of more than 90% vs 69% (patient) and 45% (kidney) for those not achieving complete remission.