Epidemiology, Impact, and Management Strategies of Anti-Glomerular Basement Membrane Disease

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Temporal associations with influenza, SARS-CoV-2 infection, and COVID-19 vaccination have been described although there is insufficient evidence to suggest causality. Anti-GBM disease accounts for approximately 20% of the cases of rapidly progressive GN cases secondary to crescentic GN, but is an uncommon cause of end-stage kidney disease. Early diagnosis by detection of circulating antibodies, increased awareness of atypical as well as complex clinical variants of the disease, and combined therapy with immunosuppression and plasma exchange has improved the prognosis of patients with this potentially fatal disease. Progress has been hampered by the rarity of anti-GBM disease, but new agents and therapeutic regimens are emerging.

Subsequent COVID-19 Prophylaxis in COVID-19 Associated Glomerulopathies

“…It is particularly important to recognize vaccine-associated GN as a clinical entity, and have awareness of management strategies due to emergence of case reports and series following the implementation of COVID-19 vaccination programs [ 3 , 6 , 8 , 36 , 37 , 38 ]. Recent systematic reviews of renal disease associated with COVID-19 vaccination showed that MCD occurred most frequently, followed by IgA nephropathy, and vasculitis [ 5 , 39 ].…”

Immunity in the balance: Fatal disseminated adenovirus infection in a patient undergoing plasma exchange and immunosuppressive chemotherapy for anti‐glomerular basement membrane disease

“…[1][2][3] Epidemiological studies have noted a bimodal age distribution, characteristically affecting males in their third decade of life with renal and pulmonary involvement, and a second peak among females in their sixth to seventh decade of life with isolated renal disease. 1,4 Most patients present with features of rapidly progressive glomerulonephritis, and a subset of patients may also develop diffuse alveolar hemorrhage. 5 The standard therapeutic regimen for anti-GBM disease involves therapeutic plasma exchange (TPE), cyclophosphamide, and corticosteroids to rapidly remove and inhibit autoantibody production and reduce organ inflammation.…”

“…As it is characterized by circulating autoantibodies targeting basement membrane antigens, it is considered an immune‐complex small‐vessel vasculitis 1‐3 . Epidemiological studies have noted a bimodal age distribution, characteristically affecting males in their third decade of life with renal and pulmonary involvement, and a second peak among females in their sixth to seventh decade of life with isolated renal disease 1,4 . Most patients present with features of rapidly progressive glomerulonephritis, and a subset of patients may also develop diffuse alveolar hemorrhage 5 …”

Isolated pulmonary anti‐glomerular basement disease in Central Western New South Wales

“…Anti-glomerular basement (anti-GBM) disease is a rare autoimmune vasculitis affecting alveolar and GBM membranes, with reported incidence of 0.4-2.8 cases per million, 1 occurring predominantly in male Caucasians, with a bimodal age distribution in the third and seventh decades. 2,3 While 80-90% of patients present with rapidly progressive glomerulonephritis, 40-60% of these cases present with coexisting pulmonary manifestations, 3,4 with isolated pulmonary manifestations seen in less than 10% of cases. 2,5 Here, we report two cases of isolated pulmonary anti-GBM disease presented to a regional hospital in Central Western New South Wales (NSW), Australia.…”

“…2,3 While 80-90% of patients present with rapidly progressive glomerulonephritis, 40-60% of these cases present with coexisting pulmonary manifestations, 3,4 with isolated pulmonary manifestations seen in less than 10% of cases. 2,5 Here, we report two cases of isolated pulmonary anti-GBM disease presented to a regional hospital in Central Western New South Wales (NSW), Australia.…”