IgA-Nephropathie und Purpura Schönlein-Henoch - ein breites Spektrum

Mani, Laila-Yasmin; Huynh-Do, Uyen; Vogt, Bruno; Ackermann, Daniel (2015). IgA-Nephropathie und Purpura Schönlein-Henoch - ein breites Spektrum. Therapeutische Umschau, 72(3), pp. 157-160. Huber 10.1024/0040-5930/a000659

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IgA nephropathy is the most common glomerulonephritis in Europe. The disease has been discovered in 1968 in Paris by Jean Berger at the Necker-Children's Hospital. Diagnosis is made by kidney biopsy and requires the presence of mesangial deposits of IgA. This form of glomerulonephritis can be seen in children and adults. In childhood, it most frequently presents within the context of Schoenlein-Henoch purpura. In adulthood, the most common form is limited to the kidney. Schoenlein-Henoch purpura can be seen in adults and manifests as a very aggressive vasculitis, usually in the context of a specific drug intake. The underlying pathophysiological concept today is an insufficient glycosylation of the IgA1 hinge region triggering the formation of autoantibodies against this site. Therapeutic options for the disease are limited. Important is optimal blood pressure control. Selected patients will profit from steroid therapy.

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