Henoch-Schönlein Vaskülitinde Şiddetli Gastrointestinal Tutulum: Vaka Sunumu

Abstract

Henoch-Schönlein purpurası HSP ile ilişkili gastrointes- tinal sistem bulguları karın ağrısından obstrüksiyona dek değişebilmektedir. Tedavide oral ya da intravenöz steroid kullanılabilmekte, yanıt alınamadığında puls metilpredni- zolon PMP , intravenöz immunglobulin ya da plazmaferez gibi tedavi seçeneklerine geçilebilmektedir. Altı yaşında erkek vaka bilateral alt ekstremitelerde palpabl purpura, bileklerde artrit, lomber ve frontal bölgelerde ağrılı yumu- şak doku ödemleri, skrotal ekimotik görünüm bulguları ile HSP tanısı aldı. İzleminde şiddetli karın ağrısı ve kanlı dışkılama yakınmaları gelişti. İki mg/kg dozunda başlanan steroid tedavisine yanıt alınamayarak PMP tedavisine geçildi. Tedavinin ilk dozunda epididimit gelişen ve üçüncü dozunda halen kanlı dışkılama izlenen vakada üçüncü doz- dan sonra yakınmalerde gerileme saptandı

Keywords

• Henoch-Schönlein purpurası,gastrointestinal sistem,puls metilprednizolon

Severe Gastrointestinal Involvement in HenochSchoenlein Purpura: Case Report

Abstract

Henoch-Schönlein purpura HSP associated gastrointesti- nal symptoms vary from abdominal pain to obstruction. Oral/intravenous steroid is the treatment of choice. In case of failure, pulse methylprednisolone PMP , intravenous immunoglobulin, plasmapheresis may be needed. A six- year-old boy was diagnosed with HSP owing to palpable purpura, arthritis, painful soft tissue edema in lumbar and frontal areas, ecchymosis in scrotum. During the follow-up, he had severe abdominal pain and bloody stool, which did not respond to steroid therapy 2 mg/kg . PMP was institu- ted. Epididymitis developed after the first dose. and he had bloody stool while receiving the third dose, but remission in complaints was observed thereafter

Keywords

• Henoch-Schoenlein purpura,gastrointestinal system,pulse methylprednisolone

References

1. Szer IS. Henoch-Schönlein purpura. Curr Opin Rheumatol ;6:25-31. http://dx.doi.org/10.1097/00002281-199401000-00005 PMid:8031676
2. Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of diffe- rent ethnic origins. Lancet 2002;360:1197-202. http://dx.doi.org/10.1016/S0140-6736(02)11279-7
3. JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis ;65:936-41. http://dx.doi.org/10.1136/ard.2005.046300 PMid:16322081 PMCid:PMC1798210
4. Acute scrotum in Henoch-Schönlein purpura: fact or fiction? Turk J Pediatr 2012;54:194-7. PMid:22734311
5. Cassidy JT, Petty RE, Laxer RM, Lindsley CB, eds. Textbook of Pediatric Rheumatology. 5th ed. Philadelphia: WB Saunders; 2005:496-507. http://dx.doi.org/10.1016/B978-1-4160-0246-8.50029-2
6. Zhang Y, Huang X. Gastrointestinal involvement in Henoch- Schönlein purpura. Scand J Gastroenterol 2008;43:1038-43. http://dx.doi.org/10.1080/00365520802101861 PMid:18609159 et al. Gastrointestinal involvement in Henoch-Schönlein syndrome: CT findings. Am J Roentgenol 1997;168:965-8. http://dx.doi.org/10.2214/ajr.168.4.9124151 PMid:9124151
7. Paradis J. Perforation of large and small bowel in Henoch- Schonlein purpura. Int J Dermatol 1997;36:361-3. http://dx.doi.org/10.1111/j.1365-4362.1997.tb03098.x PMid:9199985
8. Heuschkel RB, Murch SH. Massive gastrointestinal hae- morrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr 2007;166:915-9. http://dx.doi.org/10.1007/s00431-006-0337-3 PMid:17120033

9. Warris A, Draaisma JM. Refractory severe intestinal vascu- litis due to Henoch-Schönlein purpura: successful treatment with plasmapheresis. Acta Paediatr 2006;95:622-3. http://dx.doi.org/10.1080/08035250500509180 PMid:16825146 treatment of gastrointestinal involvement in Henoch-Schönlein purpura with plasmapheresis. Pediatr Nephrol 2008;23:2103. http://dx.doi.org/10.1007/s00467-008-0886-y PMid:18546025
10. Schoenlein purpura: pathophysiologic mechanisms, the diag- nostic value of factor XIII, and therapeutic options. Eur J Pediatr 2007;166:1199-200. http://dx.doi.org/10.1007/s00431-007-0415-1 PMid:17333275 ment of mesenteric vasculitis caused by Henoch-Schönlein purpura with methylprednisolone pulse therapy. Clin Rheumatol 2003;22:140-2. http://dx.doi.org/10.1007/s10067-002-0676-z PMid:12740680