A rare complication of IgA vasculitis: renal and intestinal ischemia successfully treated with plasmapheresis

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Türkmen Ş., Taşar S., Güzel M., SÖNMEZ H. E., Çakan M., Sözeri B.

Turkish Journal of Pediatrics, cilt.65, sa.5, ss.868-873, 2023 (SCI-Expanded) identifier identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 65 Sayı: 5
  • Basım Tarihi: 2023
  • Doi Numarası: 10.24953/turkjped.2022.935
  • Dergi Adı: Turkish Journal of Pediatrics
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, Veterinary Science Database, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.868-873
  • Anahtar Kelimeler: familial Mediterranean fever, IgA vasculitis, mesenteric ischemia, plasmapheresis, renal infarction
  • Kocaeli Üniversitesi Adresli: Evet

Özet

Background. IgA vasculitis (IgAV) is a multisystemic small vessel vasculitis and is the most common vasculitis in childhood. The characteristic findings of IgAV are palpable purpuric rash, abdominal pain, arthralgia or arthritis, and hematuria. Ischemic complications are very rare in IgAV. Thrombotic complications can be observed after a COVID-19 infection. Also in the presence of familial Mediterranean fever, IgAV may have an atypical or more severe course. Case. We present a case of IgAV complicated with renal infarction and intestinal ischemia. There was no recent or distant history of COVID-19 in the patient or family members, but the patient’s COVID-19 antibody was positive. In addition, MEFV gene analysis of the patient showed homozygous M694V mutation. The patient did not respond to enoxaparin, pulse methylprednisolone, intravenous immunoglobulin (IVIG), iloprost, and cyclophosphamide treatments. She was successfully treated with six sessions of plasmapheresis. Conclusions. Plasmapheresis seems to be an effective treatment option in IgAV-related ischemic findings that do not respond to intensive immunosuppressive therapy.