Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Pathology and Classification of Focal Segmental Glomerulosclerosis

초점성 분절성 사구체 경화증의 병리와 분류

  • Kim, Yong-Jin (Department of Pathology, Yeungnam University College of Medicine)
  • 김용진 (영남대학교 의과대학 병리학교실)
  • Received : 2012.03.14
  • Accepted : 2012.04.15
  • Published : 2012.04.30
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Abstract

Focal segmental glomerulosclerosis (FSGS) is the name of the primary glomerular disease as well as the terminology to describe the secondary phenomena of any other glomerular diseases. It is characterized by sclerosis, hyalinosis, foam cell infiltration, vacuolar change of podocytes, and halo formation in the glomerulus. Throughout the interstitium, lymphocytes infiltration, tubular atrophy and vascular changes are accompanied. Occasionally, IgM and/or C3 depositions are noted in the sclerotic areas. Electron microscopically, diffuse effacement of foot processes are seen in non-sclerotic area like minimal change disease. Podocyte injury patterns including vacuolar changes are frequently examined. Recently, Columbia group has suggested morphologic classification of FSGS and they demonstrated very good prognosis of tip lesion and poor prognosis of both collapsing and cellular types. However, the pathogenetic classification has been suggested by others; hyperfilteration, podocyte injury, genetic lesions etc. Further studies are necessary to understand and treat this disease.

초점성 분절성 사구체 경화증(focal segmental glomerulosclerosis; FSGS)은 경화증을 주 병변으로 하는 질환으로서 일차성 사구체 질환의 하나이면서 진행된 사구체 질환의 형태적 변화를 기술하는 단어로도 사용되고 있다. 사구체에는 경화증, 유리질 형성, 거품세포의 출현, 발세포의 공포화, 광륜형성 등이 보이고, 간질의 섬유화와 염증세포의 침윤, 세뇨관의 위축, 혈관의 비후 및 내막 섬유화 등을 특징으로 한다. 면역형광검사에서 부분적으로 IgM과 C3 등의 침착을 보이지만 면역관련 질환은 아니다. 전자현미경 검사에서는 발세포의 손상 현상으로 세포질 내의 공포화와 족돌기가 상실되는 것이 중요 소견이다. 2004년 표준화 된 FSGS의 분류는 과거의 형태학적 변형들을 모아서 임상과의 상관관계를 지웠다. 그 결과 tip형이 가장 예후가 좋으며, collapsing형이 가장 나쁜 것으로 알려졌다. 그러나 이 분류가 증례에 따라서는 적용하기가 애매한 경우가 많고, collapsing형을 FSGS에 분류하는 것에 대한 반론 등이 제기되고 있다. 한편, 임상적으로는 FSGS를 원인에 따라 분류하여 거꾸로 형태학적 공통점을 찾으려는 노력을 하고 있다. 사구체의 수가 적어서 일어나는 과여과로 인한 FSGS는 perihilar형이 많고 유전적 질환에 의한 것은 diffuse mesangial sclerosis가 특징인 것으로 주장되고 있다. FSGS는 이와 같이 아직도 밝혀져야 할 것이 많은 질환이며, 계속적인 연구가 이루어져야 할 필요가 있다.

Keywords

References

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