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紫斑病性腎炎における治療の選択

臨床像と、高度の蛋白尿を認める場合には、腎生検を行いその組織所見から、治療を選択する。臨床像と組織所見は関連があり、組織所見では半月体の量が予後と関連する。

IgA血管炎でみられる紫斑と免疫蛍光顕微鏡写真

a:下肢にみられた典型的なIgA血管炎の紫斑。
b:皮膚の血管壁は、IgAに対する免疫蛍光顕微鏡で陽性となる。
出典
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1: Kliegman: Nelson Textbook of Pediatrics, 21th ed. 1319. Figure 192.2

国際小児腎臓病研究班(ISKDC)による腎生検の組織学的重症度

ISKDCにより、腎生検組織所見の重症度が分類されている。特に、半月体形成の有無および程度が重要である。
出典
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1: Prognosis of Henoch-Schönlein nephritis in children.
著者: R Counahan, M H Winterborn, R H White, J M Heaton, S R Meadow, N H Bluett, H Swetschin, J S Cameron, C Chantler
雑誌名: Br Med J. 1977 Jul 2;2(6078):11-4.
Abstract/Text: All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.
Br Med J. 1977 Jul 2;2(6078):11-4.

紫斑病性腎炎の長期予後

出典
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1: Long-term follow-up of childhood Henoch-Schönlein nephritis.
著者: A R Goldstein, R H White, R Akuse, C Chantler
雑誌名: Lancet. 1992 Feb 1;339(8788):280-2.
Abstract/Text: A study of long-term outcome of 78 subjects who had had Henoch-Schönlein nephritis during childhood (at a mean of 23.4 years after onset) shows that severity of clinical presentation and initial findings on renal biopsy correlate well with outcome but have poor predictive value in individuals. 44% of patients who had nephritic, nephrotic, or nephritic/nephrotic syndromes at onset have hypertension or impaired renal function, whereas 82% of those who presented with haematuria (with or without proteinuria) are normal. 17 patients deteriorated clinically from an initial assessment in 1971; 7 of these had apparently completely recovered in 1976. 16 of 44 full-term pregnancies were complicated by proteinuria and/or hypertension, even in the absence of active renal disease. These findings indicate that childhood Henoch-Schönlein nephritis requires long-term follow-up, especially during pregnancy.
Lancet. 1992 Feb 1;339(8788):280-2.
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2: Prognosis of Henoch-Schönlein nephritis in children.
著者: R Counahan, M H Winterborn, R H White, J M Heaton, S R Meadow, N H Bluett, H Swetschin, J S Cameron, C Chantler
雑誌名: Br Med J. 1977 Jul 2;2(6078):11-4.
Abstract/Text: All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.
Br Med J. 1977 Jul 2;2(6078):11-4.

典型的症例における腎生検と免疫染色

腎生検(a)で管内増殖を認め、細胞性半月体を25%の糸球体で認めた。免疫染色(b)ではIgA、C3の沈着がみられた。
出典
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1: 花房規男先生ご提供

紫斑病性腎炎における治療の選択

臨床像と、高度の蛋白尿を認める場合には、腎生検を行いその組織所見から、治療を選択する。臨床像と組織所見は関連があり、組織所見では半月体の量が予後と関連する。

IgA血管炎でみられる紫斑と免疫蛍光顕微鏡写真

a:下肢にみられた典型的なIgA血管炎の紫斑。
b:皮膚の血管壁は、IgAに対する免疫蛍光顕微鏡で陽性となる。
出典
img
1: Kliegman: Nelson Textbook of Pediatrics, 21th ed. 1319. Figure 192.2