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<p>This case report concerns a&#xa0;4-year-old girl who presented with a&#xa0;reduced general condition, fever, hemoptysis and skin ulcers/oral aphthous ulcers. Within the last months, the girl had frequently had upper airway infections and a&#xa0;hoarse voice remained. The computed tomography (CT) scan of the thorax showed bilateral pulmonary patchy consolidations. The laboratory tests showed systemic inflammation and highly positive antineutrophil cytoplasmic antibodies (ANCA, myeloperoxidase antibodies). The diagnosis of an ANCA-associated vasculitis was made. This diagnostic group includes granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The diagnosis of ANCA-associated vasculitis is very rare in childhood. Nevertheless, this rare differential diagnosis should be considered in cases with inflammatory systemic manifestations, suspicious skin lesions and an overall reduced general condition with frequent infections to enable an early diagnosis and initiate the appropriate treatment. Pulmonary involvement is common and the extent of organ involvement (especially kidney involvement) plays a&#xa0;crucial role in the long-term prognosis. The treatment should always be discussed and carried out in conjunction with experienced centers for pediatric rheumatology.</p>

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Fieber, Hämoptysen und Hautulzera – ANCA-assoziierte Vaskulitis bei einem 4-jährigen Mädchen

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  • 发表日期：

    2026年6月22日

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  • Paula Hamm,
  • Leonie Milošev,
  • Marie-Therese Hansen,
  • Christiane Lex,
  • Claas Hinze,
  • Lars Schlotawa,
  • Matthias Kettwig

摘要

This case report concerns a 4-year-old girl who presented with a reduced general condition, fever, hemoptysis and skin ulcers/oral aphthous ulcers. Within the last months, the girl had frequently had upper airway infections and a hoarse voice remained. The computed tomography (CT) scan of the thorax showed bilateral pulmonary patchy consolidations. The laboratory tests showed systemic inflammation and highly positive antineutrophil cytoplasmic antibodies (ANCA, myeloperoxidase antibodies). The diagnosis of an ANCA-associated vasculitis was made. This diagnostic group includes granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The diagnosis of ANCA-associated vasculitis is very rare in childhood. Nevertheless, this rare differential diagnosis should be considered in cases with inflammatory systemic manifestations, suspicious skin lesions and an overall reduced general condition with frequent infections to enable an early diagnosis and initiate the appropriate treatment. Pulmonary involvement is common and the extent of organ involvement (especially kidney involvement) plays a crucial role in the long-term prognosis. The treatment should always be discussed and carried out in conjunction with experienced centers for pediatric rheumatology.