Eculizumab and Complement Activation in Anti−glomerular Basement Membrane Disease
Pravarut Nithagon, Frank B. Cortazar, Sujal I. Shah, Astrid Weins, Karen Laliberte, Anushya Jeyabalan, John L. Niles, Reza Zonozi
Abstract
Anti−glomerular basement membrane (anti-GBM) disease is characterized by pathogenic autoantibodies targeting the α-3 chain of type IV collagen located in the glomerular and alveolar basement membranes. Clinically, it causes rapidly progressive glomerulonephritis and pulmonary hemorrhage. Observational data and mechanistic rationale have largely informed current treatment strategies, including plasmapheresis, glucocorticoids, cyclophosphamide, and rituximab. However, overall and renal prognosis remain guarded, and advancements in therapy are needed.
Topics & Concepts
MedicineEculizumabRituximabPlasmapheresisGlomerular basement membraneRapidly progressive glomerulonephritisPulmonary hemorrhageBasement membraneCyclophosphamideComplement systemAutoantibodyGlomerulonephritisDiseaseImmunologyComplement membrane attack complexPathologyInternal medicineLungAntibodyKidneyChemotherapyVasculitis and related conditionsCoagulation, Bradykinin, Polyphosphates, and AngioedemaAmyloidosis: Diagnosis, Treatment, Outcomes