The Turkish Journal of Pediatrics
2023 , Vol 65 , Num 5
A rare complication of IgA vasculitis: renal and intestinal ischemia successfully treated with plasmapheresis
1 Department of Pediatric Rheumatology, University of Health Sciences, Ümraniye Training and Research Hospital, İstanbul2 Department of Pediatric Radiology, University of Health Sciences, Ümraniye Training and Research Hospital, İstanbul
3 Department of Pediatric Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye DOI : 10.24953/turkjped.2022.935 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.
Keywords : IgA vasculitis, mesenteric ischemia, plasmapheresis, renal infarction, familial Mediterranean fever