Universität zu Köln

Butzer, Sarina, Hennies, Imke, Gimpel, Charlotte, Gellermann, Jutta, Schalk, Gesa, Koenig, Sabine, Buescher, Anja K., Lemke, Anja and Pohl, Martin (2022). Early clinical course of biopsy-proven IgA vasculitis nephritis. BMC Pediatr., 22 (1). LONDON: BMC. ISSN 1471-2431

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Abstract

Background IgA vasculitis (IgAV) is the most common form of systemic vasculitis in childhood and frequently involves the kidney. A minority of patients with IgA vasculitis nephritis (IgAVN), especially those presenting with heavy proteinuria and/or kidney failure at onset, are at risk of chronic end-stage kidney disease. For deciding upon treatment intensity, knowledge of the short-term clinical course of IgAVN is needed to improve treatment algorithms. Methods For this retrospective multicenter study, the medical records of 66 children with biopsy-proven IgAVN were reviewed. Age, gender, medical history and therapeutic interventions were recorded. Laboratory data included serum creatinine, albumin, urinary protein excretion (UPE) and glomerular filtration rate (eGFR). Threshold values were determined for each parameter, full remission was defined as no proteinuria and eGFR > 90 ml/min/1.73m(2). Results Median age at onset of IgAVN was 8.9 years. 14.1% of the children presented with nephrotic syndrome, 50% had an eGFR below 90 ml/min/1.73 m(2) and 51.5% showed cellular crescents in renal histology. The treatment regimens varied notably. Forty-four patients were treated with immunosuppression; 17 patients with crescents or nephrotic syndrome were treated with corticosteroid (CS) pulse therapy. After 6 months, UPE had decreased from 3.7 to 0.3 g/g creatinine and the proportion of patients with a decreased eGFR had fallen from 50.0% to 35.5%. Thirteen children (26.5%) achieved full remission within 6 months. Conclusions In most patients with IgAVN proteinuria decreases slowly and kidney function improves, but full remission is reached only in a minority after 6 months. Persistent heavy proteinuria in the first two months rarely developed into long-term proteinuria. Therefore, decisions for more intense treatment should take into account the course of UPE over time. For a comparison of treatment effects, patient numbers were too small. Prospective, randomized controlled trials are necessary to clarify risk factors and the effect of immunosuppressive therapies.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Butzer, Sarina UNSPECIFIED UNSPECIFIED UNSPECIFIED Hennies, Imke UNSPECIFIED UNSPECIFIED UNSPECIFIED Gimpel, Charlotte UNSPECIFIED UNSPECIFIED UNSPECIFIED Gellermann, Jutta UNSPECIFIED UNSPECIFIED UNSPECIFIED Schalk, Gesa UNSPECIFIED UNSPECIFIED UNSPECIFIED Koenig, Sabine UNSPECIFIED UNSPECIFIED UNSPECIFIED Buescher, Anja K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lemke, Anja UNSPECIFIED UNSPECIFIED UNSPECIFIED Pohl, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-690480
DOI:	10.1186/s12887-022-03611-9
Journal or Publication Title:	BMC Pediatr.
Volume:	22
Number:	1
Date:	2022
Publisher:	BMC
Place of Publication:	LONDON
ISSN:	1471-2431
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language HENOCH-SCHONLEIN PURPURA; UROKINASE PULSE THERAPY; CHILDREN; METHYLPREDNISOLONE; EFFICACY; IMMUNOSUPPRESSION; NEPHROPATHY Multiple languages Pediatrics Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69048