Isabel H. * Starke Bauchschmerzen, „Purpura“ der Extremitäten

Präsentation zum Thema: "Isabel H. * Starke Bauchschmerzen, „Purpura“ der Extremitäten"—  Präsentation transkript:

1 Isabel H. * Starke Bauchschmerzen, „Purpura“ der Extremitäten Krea: 0.5 mg/dl, UEiw: 20 g/d, Ery: 300/µl Gastroduodenoskopie: Duodenalulcera Hautbiopsie: nicht beurteilbar Prednison 40 mg/d, Esomeprazol Rezidiv nach 8 Wochen S-Krea: 1.1 mg/dl (max. 1.6), UEiw: 8 g/d, ANA/ANCA/C3/IgA neg. Nierensonogramm: unauffällig Prednisolon: 80 mg/d Ramipril: 10 mg/d

2 Isabel H. * Starke Bauchschmerzen, „Purpura“ der Extremitäten Krea: 0.5 mg/dl, UEiw: 20 g/d, Ery: 300/µl Gastroduodenoskopie: Duodenalulcera Hautbiopsie: nicht beurteilbar Prednison 40 mg/d, Esomeprazol Rezidiv nach 8 Wochen S-Krea: 1.1 mg/dl (max. 1.6), UEiw: 8 g/d, ANA/ANCA/C3/IgA neg. Nierensonogramm: unauffällig Prednisolon: 80 mg/d Ramipril

3 A case report of Henoch-Schoenlein purpura nephritis associated with a postbulbar duodenal ulcer
Miura M et al. Tokai J Exp Clin Med Mar;7(2):

4 Isabel H. *18.06.1993 ? Ramipril 10 mg/d Pred 40 mg Pred 80 mg

5 Isabel H. * Mesangioproliferative Immunkomplex Nephritis (Typ IgA-Nephritis) Fokal segmentale Glomerulosklerose Fokal segmentale extrakapilläre Proliferation (12/16 Glom) Multifokaler Tubulusschaden 20% des Interstitiums

6 Histologie IgA-Nephropathie

7 Carolin S., * 11/2002 Purpura Schönlein Henoch: Petechien, Arthritis, Bauchschmerzen 12/2002 Makrohämaturie, Proteinurie 1. Nierenbiopsie: keine Glomerula, normales Interstitium progrediente Niereninsuffizienz, metabolische Azidose, Hypertonie

8 Histologie Carolin

9 Histologie Carolin

10 Histologie Carolin

11 Histologie Carolin

12 Histologie Carolin

13 Nierenfunktion Carolin S.
2. Biopsie MPred CyC HD

14 Marco E. * 17.03.1977 1988 „idiopahisches nephrotisches Syndrom“
4 Rezidive bis 05/1989 05/1989 Nierenbiopsie: IgA Nephropathie, 1/32 Glomerula mit segmentaler Sklerose, minimal vermehrte mesangiale Matrix mit Zellproliferationen, kleine Foci eines tubulären Schadens mit Fibrose Prednisolon 1 Jahr / ACE-Inhibitor Remission seit 1990

15 Histologie Marco E.

16 Marco E. * 17.03.1977 1988 „idiopahisches nephrotisches Syndrom“
4 Rezidive bis 05/1989 05/1989 Nierenbiopsie: IgA Nephropathie, 1/32 Glomerula mitn segmentaler Sklerose, minimal vermehrte mesangiale Matrix mit Zellproliferationen, kleine Foci eines tubulären Schadens mit Fibrose Prednisolon 1 Jahr / ACE-Inhibitor Remission seit 1990

17 Klinik der IgA-Nephropathie/PSHN
Nephritisches Syndrom Nephritisches Syndrom mit rapid progressiver GN Nephritisch – nephrotisches Syndrom Nephrotisches Syndrom Es gibt noch keinen validen spezifischen Parameter ! „Urin-Proteomics“ Gal-d IgA1

18 Purpura Schönlein Henoch
Leukozytoklastische Vaskulitis: Kapillaren, prä- und postkapilläre Gefäße Winter, Frühling Jungen > Mädchen, Alter 4-5 Jahre Ätiologie: unbekannt, Atemwegsinfekte, Streptokokken ? abgelagerte Immunkomplexe: IgA Symptome: Petechien, Arthritis 70%, Bauchschmerzen 50-70%

19 Prognose der IgA-N/PSHN
26-31% bei Nierenbiopsien (Erwachsene) Überlebensrate der Nieren 5 Jahre: 82% - 100% 10 Jahre: 78% - 95% 20 Jahre: 68% - 89%

20 IgA-N/PSHN Prognostisch relevante Faktoren
Mäßige bis schwere Proteinurie bei Dx Hypertonie bei Dx Höheres Alter Männliches Geschlecht Afrikanisch amerikanisch Histologie: Glomerulosklerose, Halbmonde, fokale Läsionen > 30% Glomerulosklerose/Halbmonde

21 Purpura Schönlein Henoch N. IgA - Nephritis
: J Am Soc Nephrol Jun;18(6): Epub 2007 May 18. Links Comment in: J Am Soc Nephrol Jun;18(6): Nat Clin Pract Nephrol Nov;3(11):594-5. IgACE: a placebo-controlled, randomized trial of angiotensin-converting enzyme inhibitors in children and young people with IgA nephropathy and moderate proteinuria. Coppo R, Peruzzi L, Amore A, Piccoli A, Cochat P, Stone R, Kirschstein M, Linné T. Nephrology, Dialysis and Transplantation Unit, Regina Margherita University Hospital, Turin, Italy. This European Community Biomedicine and Health Research-supported, multicenter, randomized, placebo-controlled, double-blind trial investigated the effect of an angiotensin-converting enzyme inhibitor (ACE-I) in children and young people with IgA nephropathy (IgAN), moderate proteinuria (>1 and <3.5 g/d per 1.73 m(2)) and creatinine clearance (CrCl) >50 ml/min per 1.73 m(2). Sixty-six patients who were 20.5 yr of age (range 9 to 35 yr), were randomly assigned to Benazepril 0.2 mg/kg per d (ACE-I) or placebo and were followed for a median of 38 mo. The primary outcome was the progression of kidney disease, defined as >30% decrease of CrCl; secondary outcomes were (1) a composite end point of >30% decrease of CrCl or worsening of proteinuria until > or =3.5 g/d per 1.73 m(2) and (2) proteinuria partial remission (<0.5 g/d per 1.73 m(2)) or total remission (<160 mg/d per 1.73 m(2)) for >6 mo. Analysis was by intention to treat. A single patient (3.1%) in the ACE-I group and five (14.7%) in the placebo group showed a worsening of CrCl >30%. The composite end point of >30% decrease of CrCl or worsening of proteinuria until nephrotic range was reached by one (3.1%) of 32 patients in the ACE-I group, and nine (26.5%) of 34 in the placebo group; the difference was significant (log-rank P = 0.035). A stable, partial remission of proteinuria was observed in 13 (40.6%) of 32 patients in the ACE-I group versus three (8.8%) of 34 in the placebo group (log-rank P = 0.033), with total remission in 12.5% of ACE-I-treated patients and in none in the placebo group (log-rank P = 0.029). The multivariate Cox analysis showed that treatment with ACE-I was the independent predictor of prognosis; no influence on the composite end point was found for gender, age, baseline CrCl, systolic or diastolic BP, mean arterial pressure, or proteinuria.

22 Purpura Schönlein Henoch N. IgA - Nephritis
: J Am Soc Nephrol Jun;18(6): Epub 2007 May 18. Links Comment in: J Am Soc Nephrol Jun;18(6): Nat Clin Pract Nephrol Nov;3(11):594-5. IgACE: a placebo-controlled, randomized trial of angiotensin-converting enzyme inhibitors in children and young people with IgA nephropathy and moderate proteinuria. Coppo R, Peruzzi L, Amore A, Piccoli A, Cochat P, Stone R, Kirschstein M, Linné T. Nephrology, Dialysis and Transplantation Unit, Regina Margherita University Hospital, Turin, Italy. This European Community Biomedicine and Health Research-supported, multicenter, randomized, placebo-controlled, double-blind trial investigated the effect of an angiotensin-converting enzyme inhibitor (ACE-I) in children and young people with IgA nephropathy (IgAN), moderate proteinuria (>1 and <3.5 g/d per 1.73 m(2)) and creatinine clearance (CrCl) >50 ml/min per 1.73 m(2). Sixty-six patients who were 20.5 yr of age (range 9 to 35 yr), were randomly assigned to Benazepril 0.2 mg/kg per d (ACE-I) or placebo and were followed for a median of 38 mo. The primary outcome was the progression of kidney disease, defined as >30% decrease of CrCl; secondary outcomes were (1) a composite end point of >30% decrease of CrCl or worsening of proteinuria until > or =3.5 g/d per 1.73 m(2) and (2) proteinuria partial remission (<0.5 g/d per 1.73 m(2)) or total remission (<160 mg/d per 1.73 m(2)) for >6 mo. Analysis was by intention to treat. A single patient (3.1%) in the ACE-I group and five (14.7%) in the placebo group showed a worsening of CrCl >30%. The composite end point of >30% decrease of CrCl or worsening of proteinuria until nephrotic range was reached by one (3.1%) of 32 patients in the ACE-I group, and nine (26.5%) of 34 in the placebo group; the difference was significant (log-rank P = 0.035). A stable, partial remission of proteinuria was observed in 13 (40.6%) of 32 patients in the ACE-I group versus three (8.8%) of 34 in the placebo group (log-rank P = 0.033), with total remission in 12.5% of ACE-I-treated patients and in none in the placebo group (log-rank P = 0.029). The multivariate Cox analysis showed that treatment with ACE-I was the independent predictor of prognosis; no influence on the composite end point was found for gender, age, baseline CrCl, systolic or diastolic BP, mean arterial pressure, or proteinuria.

23 Purpura Schönlein Henoch N/IgAN Aktive Nephritis bei Kindern

24 Aktive IgA-Nephropathie
Prednisolon Azathioprin Warfarin Dipyridamol * Yoshikawa; Clin J Am Soc Nephrol 1: 511–517, 2006.

25 Therapie der IgA N mit MMF
30% 50% 16 24 32 40 48 56 64 Wochen MMF % Änderung der Proteinurie Tang S Kidney International, Vol. 68 (2005), pp. 802–812

26 Therapie der IgA N mit MMF
Tang S Kidney International, Vol. 68 (2005), pp. 802–812

27 Therapie der IgA N mit MMF
Tang S Kidney International, Vol. 68 (2005), pp. 802–812

28 Isabel H. *18.06.1993 Pred 40 mg Pred 80 mg Ramipril 10
Pred 7 mg/ 48 h MMF 2 x 750 mg Ramipril 10 mg/d

29 Therapie der IgA N / PSHN
Proteinurie < 1 g/m2 * 24h Proteinurie 1-3 g/m2 * 24h Proteinurie > 3 g/m2 * 24h ACE-Inhibitoren Steroiode Steroiod Pulstherapie LZ-Steroide Zusätzliche Immunsuppression Nierenbiopsie

30 Die Geschichte geht weiter

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