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<p>Vasculitides with renal involvement are a&#xa0;heterogeneous group of systemic diseases, most of which belong to the group of small vessel vasculitides. The classical clinical presentation is nephritic syndrome with hypertension and hematuria/acanthocytes in the urine. Severe courses can manifest as rapidly progressive glomerulonephritis (RPGN) with rapid destruction of the glomeruli and loss of kidney function. The most frequent disease in this group is antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but anti-glomerular basement membrane (GBM) disease, cryoglobulinemic vasculitis and postinfectious or parainfectious glomerulonephritis can also present as RPGN. The treatment is usually nonspecific immunosuppression. An increasingly better understanding of the disease has led to the establishment of complement blockade in AAV. Another major challenge, especially in AAV, is the detection of relapses. Biomarkers for early detection have already been investigated but have not yet been included into routine clinical practice. The complexity of vasculitides and the often diverse manifestations in various organ systems require interdisciplinary care and should be carried out at a&#xa0;specialized center. With an increasingly better understanding of the immunopathogenesis of renal vasculitis, specific treatment strategies with fewer side effects for these rare diseases can be expected in the future.</p>

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Systemerkrankungen: Vaskulitis der Niere

  • 期刊论文

  • 发表日期：

    2025年12月1日

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  • Malte Hellmig,
  • Franziska von Haxthausen,
  • Christian F. Krebs

摘要

Vasculitides with renal involvement are a heterogeneous group of systemic diseases, most of which belong to the group of small vessel vasculitides. The classical clinical presentation is nephritic syndrome with hypertension and hematuria/acanthocytes in the urine. Severe courses can manifest as rapidly progressive glomerulonephritis (RPGN) with rapid destruction of the glomeruli and loss of kidney function. The most frequent disease in this group is antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but anti-glomerular basement membrane (GBM) disease, cryoglobulinemic vasculitis and postinfectious or parainfectious glomerulonephritis can also present as RPGN. The treatment is usually nonspecific immunosuppression. An increasingly better understanding of the disease has led to the establishment of complement blockade in AAV. Another major challenge, especially in AAV, is the detection of relapses. Biomarkers for early detection have already been investigated but have not yet been included into routine clinical practice. The complexity of vasculitides and the often diverse manifestations in various organ systems require interdisciplinary care and should be carried out at a specialized center. With an increasingly better understanding of the immunopathogenesis of renal vasculitis, specific treatment strategies with fewer side effects for these rare diseases can be expected in the future.