SUCCESSFUL TREATMENT WITH PLASMAPHERESIS AND IMMUNOSUPPRESSANTS IN ANCA-NEGATIVE GOOD PASTURE SYNDROME: A RARE CASE REPORT

Abstract

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Goodpasture syndrome (GPS), or Anti-glomerular basement membrane disease is a rare autoimmune disease with an annual incidence of 0.5-1.6 cases per million [1]. We report a case of cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) negative GPS disease where combined immunosuppressant and plasmapheresis treatment resulted in significant improvement of both hemoptysis and renal function. CASE PRESENTATION: A 19-year-old female with past medical history of obesity, asthma and chronic cough presented with shortness of breath, and hemoptysis of two week duration despite being treated with several courses of antibiotics for presumed recurrent pneumonia. She was found to be hypertensive on arrival (BP 163/95 mmHg). Work up revealed elevated creatinine, significant hematuria and proteinuria. CT chest demonstrated bilateral multifocal patchy infiltrates suspicious for pneumo-renal syndrome. She was empirically started on pulse dose methylprednisolone. Autoimmune workup revealed positive anti-glomerular basement membrane (anti-GBM) antibodies (36U ; normal value <20U) and negative C-ANCA. Renal biopsy showed acute necrotizing fibrocellular crescents involving more than two third of the glomeruli and linear GBM staining for IgG suggestive of anti-GBM nephritis confirming the diagnosis of GPS. Treatment with five rounds of plasmapheresis and immunosuppressive therapy with cyclophosphamide followed by extended release prednisone taper resulted in significant clinical improvement. DISCUSSION: GPS is characterized by auto reactivity to antigens in type IV collagen chains present in the glomeruli and alveolar basement membrane capillaries resulting in crescentic glomerulonephritis and diffuse pulmonary alveolar hemorrhage[2]. Additionally, ANCA is associated with GBM in 20-60% of the cases. The current treatment includes plasma exchange(PE) which involves removal of circulating anti-GBM antibodies and other mediators of inflammation, and immunosuppressants that minimize new antibody formation[3]. Although PE is effective in the treatment of GPS, the efficacy of combined immunosuppressant with PE is not well established. Our case is unique that the combined treatment with immunosuppressants and plasmapheresis resulted in a significant improvement of both hemoptysis and renal function in a ANCA negative good pasture’s disease. As GPS is associated with irreversible renal damage and high mortality rate, early and appropriate treatment may reverse the kidney damage and prevent the need for hemodialysis[2]. However, further multi-center clinical trials are required to establish its efficacy in the treatment of GBM. CONCLUSIONS: Our case highlights that PE in combination with cyclophosphamide resulted in significant improvement when compared to treatment with PE alone as reported in the literature. Early diagnosis and treatment can result in favorable outcomes in patients with GPS. Reference #1: Marques, C., et al., Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients. Front Immunol, 2019. 10: p. 1665. Reference #2: Apaydin, S., The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis. Transfus Apher Sci, 2018. 57(1): p. 8-12. Reference #3: Prendecki, M. and C. Pusey, Plasma exchange in anti-glomerular basement membrane disease. Presse Med, 2019. 48(11 Pt 2): p. 328-337. DISCLOSURES: No relevant relationships by Ioana Amzuta, source=Web Response No relevant relationships by Anupama B K, source=Web Response No relevant relationships by Japjot Chahal, source=Web Response No relevant relationships by Christopher Ramos, source=Web Response No relevant relationships by Akhila sunkara, source=Web Response