Glomerulonephritis in the Vasculitides
Glomerulonephritis in the Vasculitides
Systemic vasculitis refers to a condition of blood vessel inflammation, of which the causes are various. In a substantial number of cases, autoantibodies against neutrophil cytoplasm constituents (ANCAs) are present. The authors then refer to the systemic vasculitis as ANCA-associated systemic vasculitis. Renal disease is an unfavorable component, leading to dialysis dependency in a considerable number of patients. This review aims to summarize in brief what was reported about ANCA-associated vasculitis in the recent past. What the exact pathogenic role of ANCAs in the development of systemic vasculitis is remains uncertain, and it is still not clear how their presence leads to the histopathologic lesions called vasculitis.
Vasculitis is an inflammatory process of blood vessels, histopathologically characterized by vessel wall destruction and occlusion of the vascular lumen. Although vasculitis may be confined to a particular organ, mostly multiple organ systems are involved, a condition referred to as systemic vasculitis. Vasculitis may occur secondary to underlying diseases, such as rheumatoid arthritis and systemic lupus erythematosus, infection (eg, infectious endocarditis), or drug reactions (eg, to propylthiouracil), but it may also be primary. Most of the primary and some of the secondary vasculitides are associated with the presence of circulating antibodies directed against lysosomal constituents of neutrophils and monocytes. These antibodies are known as antineutrophil cytoplasm autoantibodies (ANCAs). Many attempts have been made to classify the systemic vasculitides properly, which is difficult because of the considerable overlap of clinicopathologic features. The most well-known proposal is that of the Chapel Hill Consensus Conference, mainly based on vessel size, but there is a call for modification. Ideally, classification systems should help to determine therapy regimens and predict for outcome. However, in clinical practice, their usefulness seems limited.
Systemic vasculitis refers to a condition of blood vessel inflammation, of which the causes are various. In a substantial number of cases, autoantibodies against neutrophil cytoplasm constituents (ANCAs) are present. The authors then refer to the systemic vasculitis as ANCA-associated systemic vasculitis. Renal disease is an unfavorable component, leading to dialysis dependency in a considerable number of patients. This review aims to summarize in brief what was reported about ANCA-associated vasculitis in the recent past. What the exact pathogenic role of ANCAs in the development of systemic vasculitis is remains uncertain, and it is still not clear how their presence leads to the histopathologic lesions called vasculitis.
Vasculitis is an inflammatory process of blood vessels, histopathologically characterized by vessel wall destruction and occlusion of the vascular lumen. Although vasculitis may be confined to a particular organ, mostly multiple organ systems are involved, a condition referred to as systemic vasculitis. Vasculitis may occur secondary to underlying diseases, such as rheumatoid arthritis and systemic lupus erythematosus, infection (eg, infectious endocarditis), or drug reactions (eg, to propylthiouracil), but it may also be primary. Most of the primary and some of the secondary vasculitides are associated with the presence of circulating antibodies directed against lysosomal constituents of neutrophils and monocytes. These antibodies are known as antineutrophil cytoplasm autoantibodies (ANCAs). Many attempts have been made to classify the systemic vasculitides properly, which is difficult because of the considerable overlap of clinicopathologic features. The most well-known proposal is that of the Chapel Hill Consensus Conference, mainly based on vessel size, but there is a call for modification. Ideally, classification systems should help to determine therapy regimens and predict for outcome. However, in clinical practice, their usefulness seems limited.
