Isabel H. *18.06.1993 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

Isabel H. *18.06.1993 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

A case report of Henoch-Schoenlein purpura nephritis associated with a postbulbar duodenal ulcer Miura M et al. Tokai J Exp Clin Med. 1982 Mar;7(2):245-50.

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

Isabel H. *18.06.1993 Mesangioproliferative Immunkomplex Nephritis (Typ IgA-Nephritis) Fokal segmentale Glomerulosklerose Fokal segmentale extrakapilläre Proliferation (12/16 Glom) Multifokaler Tubulusschaden 20% des Interstitiums

Histologie IgA-Nephropathie

Carolin S., *23.04.1995 11/2002 Purpura Schönlein Henoch: Petechien, Arthritis, Bauchschmerzen 12/2002 Makrohämaturie, Proteinurie 1. Nierenbiopsie: keine Glomerula, normales Interstitium progrediente Niereninsuffizienz, metabolische Azidose, Hypertonie

Histologie Carolin

Histologie Carolin

Histologie Carolin

Histologie Carolin

Histologie Carolin

Nierenfunktion Carolin S. 2. Biopsie MPred CyC HD

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

Histologie Marco E.

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

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

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%

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%

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

Purpura Schönlein Henoch N. IgA - Nephritis : J Am Soc Nephrol. 2007 Jun;18(6):1880-8. Epub 2007 May 18. Links Comment in: J Am Soc Nephrol. 2007 Jun;18(6):1633-4. Nat Clin Pract Nephrol. 2007 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, 10127 Turin, Italy. nefrologia@oirmsantanna.piemonte.it 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.

Purpura Schönlein Henoch N. IgA - Nephritis : J Am Soc Nephrol. 2007 Jun;18(6):1880-8. Epub 2007 May 18. Links Comment in: J Am Soc Nephrol. 2007 Jun;18(6):1633-4. Nat Clin Pract Nephrol. 2007 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, 10127 Turin, Italy. nefrologia@oirmsantanna.piemonte.it 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.

Purpura Schönlein Henoch N/IgAN Aktive Nephritis bei Kindern

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

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

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

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

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

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

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