Henoch Schönlein Purpuralı 137 Olgunun 10 Yıllık Retrospektif Değerlendirilmesi

Yıl 2011, Cilt: 9 Sayı: 3, 63 - 67, 01.12.2011

Osman Dönmez , Nihal Sargın Yıldırım Oğuzhan Durmaz

Öz

Giriş: Bu çalışmamızda Uludağ Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Çocuk Nefroloji Bilim Dalında 1999-2009 yılları arasında en az bir yıllık izlemi olan HSP tanısı alan çocuklar laboratuar, klinik ve izlem sonuçları ile değerlendirildi. Gereç ve Yöntem: Uludağ Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Pediatrik Nefroloji Bilim Dalında 1999-2009 yılları arasında en az bir yıllık izlemi olan HSP’li çocukların epidemiyolojik, klinik, laboratuar bulguları ve tedavi bilgileri dosya kayıtları incelenerek elde edildi. Bulgular: Çalışmaya alınan 137 HSP’li hastanın 48’i kız %35 , 89’u erkek %65 idi. Tüm olgularda nontrombositopenik purpurik döküntüler gözlendi. Seksen bir %59,1 hastada Eklem tutulumu, 4 %2,9 hastada pulmoner tutulum, 99 %72,3 hastada gastrointestinal GİS tutulum mevcuttu. Otuz hastaya böbrek biyopsisi yapıldı ve hastaların 16’sında %11,7 evre 1, 2’sinde %1,5 evre 2A, 3’ünde %2,2 evre 2B, 3’ünde %2,2 evre 3A, 5’inde %3,6 evre 3B ve 1’inde %0,7 evre 4 tutulum saptandı. Meadow sınıflamasına göre hastalar klinik açıdan değerlendirildiğinde en sık mikroskobik hematüri 55 %40,1 görüldü. Onbeş %10,9 hastada akut nefritik sendrom saptanırken 7 %5,19 hastada da nefrotik sendrom, 4 %2,9 hastada Ailevi Akdeniz Ateşi, 1 %0,7 hastada Poliarteritis Nodosa saptandı. Laboratuvar bulgularında 60 %43,8 hastada lökosit >10.000 saptandı. Hastaların 72’si %52,6 herhangi bir tedavi uygulanmadan izlendi, diğer hastalarda hastalığın ağırlığına göre steroid, NSAİİ veya immünsüpresif tedavi uygulandı. Takipteki hastaların 16’sında %11,7 nüks saptandı. Sonuç: Henoch Schönlein purpurası spontan iyileşebilen, ancak kimi zaman ölümle sonuçlanabilecek kadar ağır komplikasyonlara yol açabilen ve kimi zaman da atipik seyirle gidebilen bir hastalıktır. Akut dönemde hastaların komplikasyonlar açısından çok yakın izlemi ve olabilecek nüksler ve özellikle böbrek tutulumu açısından uzun süreli izlemleri son derece gereklidir. Gün cel Pe di at ri 2011; 9: 63-7

Anahtar Kelimeler

Henoch-Schönlein purpurası, vaskülit, çocuklar

A Ten-Year Retrospective Evaluation of 137 Patients with Henoch Schonlein’s Purpura

Öz

Introduction: In this study, we have evaluated laboratory, clinical characteristics, and results of Henoch Schonlein’s purpura HSP patients diagnosed in Uludag University Hospital Pediatric Nephrology outpatient clinic between 1999 and 2009. Material and Methods: The laboratory, clinical characteristics, and results of the patients were derived from hospital records. Results: Of 137 HSP patients, 48 35% were girls, 89 65% were boys. Nonthrombocytopenic purpura was present in all patients. Joint involvement was seen in 81 59.1% patients. Pulmonary involvement was seen in 4 2.9% patients. Gastrointestinal system involvement was seen in 99 72.3% patients. Thirty patients underwent a kidney biopsy. In 16 11.7% patients grade I, in 2 1.5% patients grade 2A, in 3 2.2% patients grade 2B, in 3 2.2% patients grade 3A, in 5 3.6% patients grade 3B, and in 1 0.7% patient grade 4 renal involvement was present. When we evaluated the patients according to Meadow’s classification, microscopic hematuria was the most commonly seen finding in 55 %40.1 patients. Acute nephritic syndrome was present in 15 10.9% patients, nephrotic syndrome in 7 5.19% patients, familial Mediterranean fever in four 4 2.9% patients and poliarteritis nodosa in one 0.7% patient. On laboratory findings, leukocyte >10000/ml was seen in 60 43.8% patients. Seventy two 52.6% patients were followed without treatment. Other patients were treated with steroids, NSAID and immunosuppressive therapy according to the severity of diseases. In 16 11.7% patients recurrence was seen. Conclusion: HSP is a disease that can spontaneously resolve, but sometimes can be present with complications leading to death. Some patients show atypical manifestations. While acutely diagnosed patients should be inspected for complications, the patients should be closely monitored for recurrences and renal involvement after acute period. Jo ur nal of Cur rent Pe di at - rics 2011; 9: 63-7

Anahtar Kelimeler

Henoch-Schönlein purpura, vasculitis, children

Kaynakça

• 1. Heberden W. Commentaries on the history and cure of disease. Birmingham: Division of Griphon Editions Ltd; 1982.p.395-7.
• 2. Schönlein JL. Allgemenie und specielle Pathologie und Therapie. 3 rd ed. Wurzburg: im Literatur-Comptoir; 1837.
• 3. Cassidy JT, Petty RE. Leukocytoclastic vasculitis. In:Cassidy JT, Petty RE editors. Textbook of Pediatric Rheumatology. 5 th ed. Philadelphia: W.B. Saunders Co.; 2005.p.496-501.
• 4. Michael LM, Lauren MP. Vasculitis syndromes. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed, Philadelphia: WB Saunders Co, 2004: 826-9.
• 5. Bagga A, Dillon MJ. Leukocytoclastic vasculitis. In: Cassidy JT, Petty RE, eds. Textbook of Pediatric Rheumatology. Philadelphia: W.B. Saunders; 2001:569-79.
• 6. Nielsen HE. Epidemiology of Schönlein Henoch purpura. Acta Pediatr Scand 1988;77:125-31.
• 7. Peru H, Soylemezoglu O, Bakkaloglu SA, Elmas S, Bozkaya D, Elmaci AM, et al. Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period. Clin Rheumatol 2008;27:1087-92.
• 8. Lahita RG. Influence of age on Henoch Schönlein purpura. Lancet 1997;350:1116-7.
• 9. Saulsbury FT. Henoch Schönlein purpura in children: report of 100 patients and review of the literature. Medicine 1999;78:395-409.
• 10. Dolezalova P, Telekesova P, Nemcova D, Hoza J. Incidence of vasculitis in children in the Czech republic:2-year prospective epidemiology survey. J Rheumatol 2004;31:2295-9.
• 11. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F. Henoch Schonlein Purpura in Childhood: Epidemiological and Clinical Analysis of 150 Cases Over a 5-year Period and Review of Literature. Semin Arthritis Rheum 2005;35:143-53.
• 12. Aydın M, Demirol M, Kurt A, Kurt NAC, Yılmaz E. The evaluation of the our patients with Henoch-Schönlein purpura. Çocuk Dergisi 2005;5:249-53.
• 13. Cakır M, Orhan F, Mungan I, Sonmez FM, Aslan Y, Kalyoncu M, et al. Henoch-Schonlein purpura in North-eastern Turkey. Ann Trop Paediatr 2006;26:59-65.
• 14. Garcia-Porrua C, Calvino MC, Llorca J, Couselo JM, González￾Gay MA. Henoch-Schönlein purpura in children and adults: clinical differences in a defined population. Semin Arthritis Rheum 2002;32:149-56.
• 15. Calvino MC, Llorca J, Garcia-Porrua C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from Northwestern Spain. Medicine (Baltimore) 2001;80:279-90.
• 16. Scharer K, Krmar R, Qerfeld U, Ruder H, Waldherr R, Schaefer F. Clinical outcome of Schönlein-Henoch purpura nephritis in children. Pediatr Nephrol 1999;13:816-23.
• 17. Makker SP. Glomerular diseases. In: Kher KK, Makker SP, editors: Clinical Pediatric Nephrology. 1st. ed. Singapore: McGraw-Hill, Inc. 1992.p.175-277.
• 18. Robson WLM, Leung AKC. Henoch- Schönlein purpura. Adv Pediatr 1994;41:163-94.
• 19. Al-Sheyyab M, El-Shanti H, Ailouni S, Sawalha D, Daoud A. The clinical spectrum of Henoch-Schönlein purpura in infants and young children. Eur J Pediatr 1995;154:569-72.
• 20. Soylu A, Kavukçu S. Henoch-Schönlein purpura in childhood: Pathophysiology, diagnosis and treatment. SSK Tepecik Hast Derg 2004;14:71-81.
• 21. Athreya BH. Vasculitis in Children. Pediatr Clin North Am 1995;42:1239-61.
• 22. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schönlein-Henoch Syndrome). AMA J Dis Child 1960;99:833-54.
• 23. Watts RA, Scott DG. Epidemiology of the vasculitides. Semin Respir Crit Care Med 2004;25:455-64.
• 24. Ebert EC. Gastrointestinal manifestations of Henoch-Schonlein purpura. Dig Dis Sci 2008;53:2011-9.
• 25. Schwab J, Benya E, Lin R, Majd K. Contrast enema in children with Henoch-Schonlein purpura. J Pediatr Surg 2005;40:1221-3.
• 26. Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients. Acta Paediatr 2004;93:1427-31.
• 27. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, et al. Henoch-Schonlein purpura in childhood: Epidemiological and clinical analysis of 150 cases over a 5-year period and review of Literature. Semin Arthritis Rheum 2005;35:143-53.
• 28. Kaku Y, Nohara K, Honda S. Renal involvement in Schonlein￾Henoch Purpura: a multivariate analysis of prognostic factors. Kidney Int 1998;53:1755-9.
• 29. Sohar E, Gafni J, Pras M, Heler H. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med 1967;43:227-53.
• 30. Ozdogan H, Arisoy N, Kasapcapur O, Sever L, Caliskan S, Tuzuner N, et al. Vasculitis in familial Mediterranean fever. J Rheumatol 1997;24:323-7.
• 31. Huber AM, King J. McLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch schonlein purpura. BMC Med 2004;2:7.