INTRODUCTION
Rapidly Progressive Glomerulonephritis (RPGN) is a life-threatening condition often presenting with acute renal failure and characterized by a rapid decline in kidney function. It is a pathologic diagnosis where there is extensive glomerular crescent formation (i.e. >50% of sampled glomeruli contain crescents). Dual antibody positivity, particularly for anti-GBM and ANCA (Anti-Neutrophil Cytoplasmic Antibodies), is a rare and complex presentation that poses significant diagnostic and therapeutic challenges. Here we try to address such challenges through this case report of an elderly woman with dual antibody positive.
Methods:
METHODS
Case Presentation
We report a 55-year-old woman who presented with severe, rapidly progressive renal failure. Co-morbidities include diabetes, hypertension and hypothyroidism for which she was on treatment. Routine laboratory tests showed a creatinine value of 7.3 g/dL with a baseline creatinine of 1.5 g/dL 2 months back. Her initial symptoms included generalized weakness, anorexia, GI symptoms, oliguria, and edema. Laboratory investigations revealed dual positivity for anti-GBM and ANCA antibodies. Routine urine examination showed 20-22 RBCs/hpf with 1+ proteinuria. Imaging showed loss of corticomedullary differentiation, HRCT Thorax- COAD changes with pulmonary hypertension and renal biopsy confirmed the diagnosis of RPGN with features suggestive of both anti-GBM and ANCA-associated glomerulonephritis. The patient was promptly treated with methylprednisolone pulse followed by high dose corticosteroids and cyclophosphamide. This multi-faceted approach aimed to address the dual pathology and mitigate kidney damage. Supportive care, including renal replacement therapy, was also initiated.
Results:
Results
Following intensive treatment, the patient showed a significant improvement in renal function and clinical symptoms. Although renal function stabilized but she achieved only partial remission. Follow-up for next 6 months revealed sustained renal function and no recurrence of severe symptoms.
This case illustrates the challenge in treatment of double antibody-positive RPGN in an elderly female with no previous renal disease. The patient was found to be positive for anti-GBM antibody and MPO-ANCA. Treatment options include plasmapheresis, corticosteroids and immunosuppression. In this case, we treated the patient with pulse steroids for 3 days as the initial induction agent, followed by maintenance dose of oral cyclophosphamide and oral steroids. The follow up period for next 6 months, the patient did well on medications and creatinine was stabilized between between 1.9 and 2.7 g/dL. Long term follow of the patient is essential for the patient for the occurence of relapse or complications.
Conclusions:
Conclusion
This case highlights the complexities of managing dual antibody-positive RPGN and underscores the importance of a tailored, aggressive treatment strategy. Early recognition and intervention are crucial for improving outcomes in such challenging cases. Due to their mixed disease profile, patients require intensive early treatment for anti GBM illness as well as ongoing immunosuppression and close monitoring for relapse risk in the case of AAV. The goal of treatment should be to address the simple fact that there is anti-GBM positive, regardless of the results of IF staining. To further understand the mechanism underlying this uncommon illness process and to develop effective treatment plans, more research is required.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.