수막구균 뇌수막염과 보체 결핍이 동반된 막증식성 사구체신염 1례
A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis
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저자들은 C3의 감소와 육안적 혈뇨를 보인 환아에서 현미경적 혈뇨가 지속되면서 8주 후 시행한 검사에서도 C3의 감소가 지속되어 MPGN을 의심하고 신생검 예정이었다. 추적 관찰 중 수막구균 뇌수막염이 동반되었고, C3와 C5의 지속적인 감소와 C7과 C9의 일시적인 감소를 확인하였으며, 신생검에서 1형 MPGN으로 확진되었다. 본 증례의 아쉬운 점은 nephritic factor의 존재나 factor H의 결핍을 입증하여야 하나 아직까지 국내에서는 시행되지 않아 확인은 하지 못하였다. 앞으로 국내에서도 nephritic factor나 다양한 보체계(complement profile)에 대한 검사들이 시행되어야 할 것이다. 이제까지 국내에서는 후기 보체 결핍에 의하여 수막구균 뇌수막의 발생 증례 보고는 2004년 이후 여러 차례 있었지만, MPGN 환자에서 수막구균의 감염은 국내에 보고된 바가 없다. 본 증례를 통해 저보체혈증이 지속되는 일부의 MPGN 환자에서는 수막구균 감염 위험성이 존재하며, nephritic factor 검사와 함께 후기 보체 결핍에 대한 검사가 필요하다고 생각한다. 또한 수막구균성 뇌수막염에 이환된 모든 환자에 대해서 보체 검사를 시행하고 이런 환자에서 주기적인 소변 검사를 통하여 이상이 있을시 MPGN의 가능성을 고려하여 신생검을 고려하여야 할 것이다.
Abstract
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.
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