RESUMEN
The complement system is recognized as a major pathogenic or contributing factor in an ever-growing number of diseases. In addition to inherited factors, autoantibodies to complement proteins have been detected in various systemic and organ-specific disorders. These include antibodies directed against complement components, regulators and receptors, but also protein complexes such as autoantibodies against complement convertases. In some cases, the autoantibodies are relatively well characterized and a pathogenic role is incurred and their detection has diagnostic value. In other cases, the relevance of the autoantibodies is rather unclear. This review summarizes what we know of complement specific autoantibodies in diseases and identifies unresolved questions regarding their functional effect and relevance.