Goodpasture syndrome and anti-glomerular basement membrane disease

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a rare life-threatening small vessel vasculitis that typically affects the capillaries of kidneys and lungs, with most of patients developing rapidly progressive crescentic glomerulonephritis, and 40%-60% concomitant alveolar haemorrhage. It is caused by the deposition in alveolar and glomerular basement membrane of circulating autoantibodies directed against antigens intrinsic to the basement membrane. The exact mechanism that induces the formation of autoantibodies is unknown, but probably environmental factors, infections or direct damage to kidneys and lungs may trigger the autoimmune response in genetically susceptible individuals. Initial therapy includes corticosteroids and cyclophosphamide to prevent autoantibodies production, and plasmapheresis to remove the circulating autoantibodies. Good renal outcomes may be achieved by a prompt treatment initiation. However, when patients present with severe renal failure requiring dialysis or with a high proportion of glomerular crescents at biopsy, renal outcomes are bad. Relapses are rare and when renal involvement is present, the suspect of concomitant diseases, such as ANCA-associated vasculitis and membranous nephropathy, should be raised. Imlifidase is showing promising results, which if confirmed will cause a paradigm shift in the treatment of this disease.

Complement-Mediated Thrombotic Microangiopathy in a Patient With Antiphospholipid Syndrome and Anti-glomerular Basement Membrane Antibodies

“…When the patient first presented to the ED, it was presumed that the elevated creatinine was the result of anti-GBM disease and presumptive treatment was started immediately due to the potential for alveolar hemorrhage and rapid progression to renal failure [ 6 ]. It is thought that the development of anti-GBM disease may be preceded by the production of autoantibodies triggered by an unknown stimulus such as pulmonary irritation or kidney injury, and prompt intervention is warranted for the optimization of patient outcomes [ 7 ].…”

“…Anti-GBM disease, previously known as Goodpasture syndrome, is a life-threatening form of vasculitis in which IgG autoantibodies bind to the alpha-3 chain of type IV collagen in alveolar and glomerular basement membranes [ 6 , 7 ]. It typically presents with hemoptysis resulting from alveolar hemorrhage in conjunction with rapidly progressive glomerulonephritis that can result in renal failure if untreated [ 6 ]. Anti-GBM disease has previously been documented in conjunction with thrombocytopenia due to immune thrombocytopenia (ITP) [ 8 ] and with TTP [ 1 , 9 , 10 ].…”

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

“…Hemoptysis is more common in younger patients with anti-GBM disease and concurrent severe renal and pulmonary symptoms at presentation. In contrast, patients over 50 years of age usually present with glomerulonephritis only and experience a less severe disease course [7].…”

“…Goodpasture syndrome, or anti-glomerular basement membrane (anti-GBM) disease, manifests as an autoimmune condition in which antibodies target epitopes of an autoantigen found in collagen IV, a key component of kidney and lung basement membranes [7]. This article offers an updated overview of the pathogenetic mechanisms involved in the pulmonary manifestations of primary small-cell vasculitis, namely AAV and anti-GBM disease.…”

Unleashing the power of complement activation: unraveling renal damage in human anti-glomerular basement membrane disease

“…About 40%-60% of patients experience pulmonary inflammation and injury, with pulmonary hemorrhage being the initial symptom in severe cases. Failure to intervene promptly can result in respiratory distress and pose a life-threatening risk ( 23 – 25 ). It is reassuring to note that this percentage has been trending downward in recent studies (15%-35%) due to early detection and early intervention of the disease ( 3 , 4 , 26 ).…”