ORIGINAL ARTICLE

Role of mesangial fibrinogen deposition in the pathogenesis of crescentic Henoch-Schönlein nephritis in children

J I Shin¹, J M Park¹, Y H Shin¹, J S Lee¹ and H J Jeong²

¹ The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
² The Institute of Kidney Disease, Department of Pathology, Yonsei University College of Medicine, Seoul, Korea

Correspondence to:
Correspondence to:
Professor J S Lee
134 Shinchon-Dong, Seodaemun-Ku, 120-752, CPO Box 8044, Department of Paediatrics, Yonsei University College of Medicine, Seoul, Korea; jsyonse{at}yumc.yonsei.ac.kr

Aims: To clarify the role of mesangial fibrinogen deposition in crescentic Henoch-Schönlein nephritis (HSN).

Methods: A retrospective analysis of 21 children with HSN treated with immunosuppressants. Serial renal biopsies were performed before and after treatment. They were divided into two groups according to the immunofluorescent course of fibrinogen deposition: group I (n = 9), no or decreased deposition; group II (n = 12), persistent or increased deposition.

Results: There were no differences between the two groups in renal manifestations or laboratory and histological findings at presentation. However, the activity index after immunosuppressive treatment was significantly decreased in group I (mean, 7.9 (SEM, 0.7) v 2.9 (0.4); p = 0.008) and unchanged in group II (mean, 6.8 (SEM, 0.3) v 6.0 (2.1)). The chronicity index was unchanged in group I, but increased in group II (mean, 0.8 (SEM, 0.3) v 1.8 (0.3); p = 0.02). Univariate analysis revealed that the only factor significantly related to persistent or increased fibrinogen deposition was age more than 9 years (p = 0.03). Furthermore, the intensity of fibrinogen deposition at the second biopsy correlated positively with the age at onset (R² = 0.306; p = 0.009) and changes in the percentage of crescents (post-treatment crescents (%) minus pretreatment crescents (%)) correlated positively with the intensity of fibrinogen deposition at the second biopsy (R² = 0.193; p = 0.046).

Conclusions: This study indicates that fibrinogen deposition has an important role to play in renal injury of crescentic HSN and reflects persistent severe histological activity.

Abbreviations: IF, immunofluorescent; ISKDC, International Study of Kidney Disease in Children; HSN, Henoch-Schönlein nephritis

Keywords: crescentic Henoch-Schönlein nephritis; fibrinogen deposition; serial renal biopsy

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