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ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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1/2020
vol. 95
 
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abstract:
Case report

Atypical IgA-associated vasculitis – case reports

Julia Mirecka
1
,
Małgorzata Stańczyk
1, 2
,
Marcin Tkaczyk
1, 3

  1. Department of Paediatrics, Immunology, and Nephrology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
  2. Department of Paediatrics, Preventive Cardiology and Immunology of Developmental Age, Medical University of Lodz, Poland
  3. Division of Didactics in Paediatrics, Medical University of Lodz, Poland
Pediatr Pol 2020; 95 (1): 56–60
DOI: https://doi.org/10.5114/polp.2020.94935
Online publish date: 2020/03/31
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Introduction
IgA-associated vasculitis (IgA vasculitis, IgAV), formerly known as Henoch-Schönlein purpura, is the most common systemic inflammation of the small vessels in children. The disease most often affects the skin, joints, digestive tract and kidneys. It is usually characterised by a mild and self-limiting course and full recovery within a few weeks. The paper presents the cases of patients with severe and complicated IgAV.

Case report
In both cases, the kidneys were affected by the disease. The first patient had rare necrotic skin changes and splenic infarction not previously described. The second patient developed hypertension and nephropathy with nephrotic proteinuria.

Conclusions
IgA-associated vasculitis can have a severe course and produce rare complications. Patients may require non-standard individualised management as well as long-term clinical observation to monitor renal function parameters and complications of the treatment.

keywords:

vasculitis in children, nephropathy, necrotic skin lesions, splenic infarction