| H01767 | |
| H番号 | H01767 |
| 名称 | 紫斑病性腎炎 |
| 概要 | Henoch-Schonlein purpura nephritis (HSPN) is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Generally, the prognosis of Henoch-Schonlein purpura [DS:H01584] in children is favorable, but there are severe complications, one of which is renal involvement. HSPN is mainly characterized by acute episodes of glomerular inflammation with endocapillary and mesangial proliferation, fibrin deposits, and epithelial crescents that can heal spontaneously or lead to chronic lesions. HSPN and IgA nephropathy [DR:H01581] are currently considered related diseases resulting from glomerular deposition of aberrantly glycosylated IgA1. Although both nephritides present with similar histological findings and IgA abnormalities, they display pathophysiological differences with important therapeutic implications. Although there have been few evidence-based treatment strategies for HSPN, several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity. |
| カテゴリ | 免疫系疾患; 泌尿器系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | - |
| コメント | - |
| リンク | ICD-11: 4A44.92 MF8Y ICD-10: N02.9 |
| 文献 | PMID:21393485 著者 Davin JC タイトル Henoch-Schonlein purpura nephritis: pathophysiology, treatment, and future strategy. 雑誌 Clin J Am Soc Nephrol 6:679-89 (2011) DOI:10.2215/CJN.06710810 PMID:25557596 著者 Chen JY, Mao JH タイトル Henoch-Schonlein purpura nephritis in children: incidence, pathogenesis and management. 雑誌 World J Pediatr 11:29-34 (2015) DOI:10.1007/s12519-014-0534-5 PMID:25072122 著者 Davin JC, Coppo R タイトル Henoch-Schonlein purpura nephritis in children. 雑誌 Nat Rev Nephrol 10:563-73 (2014) DOI:10.1038/nrneph.2014.126 |