The pathogenic mechanism of C3 glomerulopathy and its countermeasures / 解放军医学杂志

C3 glomerulopathy is a series of diseases of glomeruli mediated by abnormal activation of alternative complement pathway. A series of researches have revealed in recent years that there are diversity and multiplicity of pathogenic mechanism in the pathogenesis of C3 glomerulopathy. The pathogenic mechanism of C3 glomerulopathy may be different in different individuals and types of disease. Congenital genetic defects and/or acquired autoantibodies may be found in the same individual. Individualized therapy should be given to individual patient in order to target different pathogenic mechanisms. Chinese herbal medicine, Tripterygium wilfordii, shows promise as a potential therapeutic agent for C3 glomerulopathy. The pathogenic mechanism and countermeasures for C3 glomerulopathy have been reviewed in present paper.

A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called "nephritic factor"(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

A Case of Postinfectious Glomerulonephritis Following Meningococcal Meningitis / 대한신장학회잡지

A 21-year-old male was presented with sudden headache, fever, petechiae and neck stiffness. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics. However the patient developed generalized edema, oliguria, azotemia, and heavy proteinuria in the recovery phase of illness. Low serum C3 level was also noted. A kidney biopsy was performed and showed the features of postinfectious glomerulonephritis and typical subepithelial humps on electron-microscopic examination. His symptoms and laboratory findings were improved, and C3 level returned to normal range after conservative treatment. We suggest that a complement deficiency should be ruled out in patients of glomerulonephritis developed during the recovery phase of meningococcal meningitis. C3 nephritic factor detection and renal biopsy should be carefully considered in these patients.