Monoclonal Antibodies for the Treatment of the C3 Glomerulopathies

[摘要] Our understanding of glomerular disease has been steadily progressing from pattern-based histologic classification and empirical immunosuppressive treatments to better molecular characterization of disease and, albeit more slowly, to more targeted therapies. The group of diseases collectively known as membranoproliferative GN (MPGN) has recently been the beneficiary of such a step forward (for review, see refs. 1–3). This histopathological pattern of MPGN can variably result from infection, autoimmune and rheumatologic disease, or monoclonal gammopathy; in such cases, both immunoglobulins and complement factor 3 (C3) are observed on immunofluorescence (IF) of the biopsy specimen. Another major subgroup of MPGN, largely consisting of the two disorders dense deposit disease (DDD; previously known as MPGN type II) and C3 GN (C3GN), arise from dysregulation of the alternative complement pathway. In contrast to the former subgroup of immune complex– or monoclonal immunoglobulin-associated MPGN, these C3 glomerulopathies are distinguished by the presence of C3, but not immunoglobulin, by IF.

[学科分类] 泌尿医学

[关键词] Bone marrow necrosis;Sickle cell disease;Hyperhemolysis syndrome