Ayten YAZICI, Ayşe ÇEFLE, Çiğdem Vural, Fatma Tuncer, Andaç Komaç

Nefrotik Düzeyde Proteinüri ile Prezente olan Henoch-Schönlein Purpurası (IgA vasküliti)

Henoch-Schönlein purpurası (HSP) çocukluk döneminin en sık görülen vaskülitidir. Erişkin çağda daha nadir görülmektedir. İmmunglobulin A (IgA) depolanması ile karakterize olup, IgA vasküliti (IgAV) olarak da isimlendirilmektedir. Bu yazıda, erişkin yaşta steroid tedavisine yanıtsız nefrotik düzeyde proteinüri ile IgAV tanısı alan olgu sunulmuştur. Tipik döküntü, gastrointestinal ve renal tutulum ile prezente olan hastaya steroid ve azatiyoprin tedavisine rağmen proteinürinin artması üzerine aylık siklofosfamid infüzyonu uygulanmıştır. Üçüncü kür sonrası proteinürisi belirgin olarak gerileyen hastanın tedavisine metilprednizolon ve azatiyoprin ile devam edilmiştir. HSP’nin erişkinlerde kabul gören bir tedavi yaklaşımı yoktur. Böbrek tutulumu büyük çocuklarda ve erişkinlerde daha sık olup morbiditenin ve mortalitenin en önemli belirleyicisidir. Bu makalede erişkin yaşta renal tutulum ile başvuran bir hastaya yaklaşımımızı paylaşmayı amaçladık. Anahtar K

Henoch-Schonlein Purpura (IgA Vasculitis) Presenting with Proteinuria at the Nephrotic Level

Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood. It is less common in adulthood. Charactarized by the storage of immunglobulin A (IgA), and its also called IgA vasculitis (IgAV). In this article, adult age case diagnosed as IgAV, with proteinuria at nephrotic level unresponsive to steroid treatment, is presented. The patient, who presented with typical rash, gastrointestinal and renal involvement, was administered monthly cyclophosphamide infusion due to increasing proteinuria despite steroid and azathioprine treatment. After the third cycle, the patients proteinuria significantly regressed and her treatment was continued with methylprednisolone and azathioprine. HSP, has no accepted treatment approach in adults. Renal involvement is more common in older children and adults, and it's the most important determinant of morbidity and mortality. In this article, we aimed to share our approach to a patient who presented with renal involvement at an adult age.

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