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Pediatr Nephrol. 2018 Feb;33(2):277-286. doi: 10.1007/s00467-017-3794-1. Epub 2017 Oct 5.

Presentation of pediatric Henoch-Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing.

Author information

1
Department of Pediatric Kidney, Liver and Metabolic Diseases, Children's Hospital, Hannover Medical School, Hannover, Germany.
2
Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center, University of Freiburg Faculty of Medicine, University of Freiburg, Mathildenstrasse 1, 79106, Freiburg, Germany.
3
Department of Pediatric Nephrology, Charité University Hospital, Berlin, Germany.
4
Department of Pediatrics, Klinikum Links der Weser, Bremen, Germany.
5
Children's Hospital Dresden, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
6
Children's Hospital, University of Leipzig, Leipzig, Germany.
7
Department of Pediatrics II, Children's Hospital, University of Duisburg-Essen, Essen, Germany.
8
KfH Center of Pediatric Nephrology, Clementine Children's Hospital, Frankfurt, Germany.
9
Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
10
Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Heidelberg, Germany.
11
Children's Hospital, Städtisches Klinikum, Karlsruhe, Germany.
12
Pediatric Nephrology Unit, University Children's Hospital, Zurich, Switzerland.
13
Department of Pediatrics, University Hospital of Cologne, Cologne, Germany.
14
University Children's Hospital, Jena, Germany.
15
KfH Center of Pediatric Nephrology, St Georg Hospital, Leipzig, Germany.
16
Department of Pediatrics, Division of Pediatric Nephrology, Mainz University Medical Center, Mainz, Germany.
17
KfH Center of Pediatric Nephrology, University Hospital Marburg, Marburg, Germany.
18
KfH Center of Pediatric Nephrology, Children's Hospital Memmingen, Memmingen, Germany.
19
University Children's Hospital Münster, Münster, Germany.
20
University Children's Hospital, Rostock, Germany.
21
University Children's Hospital, Ulm, Germany.
22
Children's Hospital, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
23
Department of Pediatrics II, Children's Hospital Augsburg, Augsburg, Germany.
24
Children's Hospital, Klinikum Esslingen, Esslingen am Neckar, Germany.
25
Krankenhaus St Elisabeth und St Barbara, Klinik für Kinder- und Jugendmedizin, Halle (Saale), Germany.
26
Department of Pediatrics, Bethanien Hospital, Moers, Germany.
27
Nephropathology Section, Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
28
Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center, University of Freiburg Faculty of Medicine, University of Freiburg, Mathildenstrasse 1, 79106, Freiburg, Germany. martin.pohl@uniklinik-freiburg.de.

Abstract

BACKGROUND:

This study correlates the clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) with findings on initial renal biopsy.

METHODS:

Data from 202 pediatric patients enrolled in the HSPN registry of the German Society of Pediatric Nephrology reported by 26 centers between 2008 and 2014 were analyzed. All biopsy reports were re-evaluated for the presence of cellular crescents or chronic pathological lesions (fibrous crescents, glomerular sclerosis, tubular atrophy >5%, and interstitial fibrosis >5%).

RESULTS:

Patients with HSPN with cellular glomerular crescents were biopsied earlier after onset of nephritis (median 24 vs 36 days, p = 0.04) than those without, whereas patients with chronic lesions were biopsied later (57 vs 19 days, p < 0.001) and were older (10.3 vs 8.6 years, p = 0.01) than those without. Patients biopsied more than 30 days after the onset of HSPN had significantly more chronic lesions (52 vs 22%, p < 0.001), lower eGFR (88 vs 102 ml/min/1.73m2, p = 0.01), but lower proteinuria (2.3 vs 4.5 g/g, p < 0.0001) than patients biopsied earlier. Children above 10 years of age had lower proteinuria (1.98 vs 4.58 g/g, p < 0.001), lower eGFR (86 vs 101 ml/min/1.73m2, p = 0.002) and were biopsied significantly later after onset of nephritis (44 vs 22 days, p < 0.001) showing more chronic lesions (45 vs 30%, p = 0.03). Proteinuria and renal function at presentation decreased with age.

CONCLUSIONS:

In summary, we find an age-dependent presentation of HSPN with a more insidious onset of non-nephrotic proteinuria, impaired renal function, longer delay to biopsy, and more chronic histopathological lesions in children above the age of 10 years. Thus, HSPN presents more like Immunoglobulin A (IgA) nephritis in older than in younger children.

KEYWORDS:

Children; Glomerulosclerosis; Henoch–Schönlein purpura; Histopathology; IgA Nephritis; Kidney biopsy

PMID:
28983704
DOI:
10.1007/s00467-017-3794-1
[Indexed for MEDLINE]

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