İmmünglobülin A Vaskülitinde Gastrointestinal Sistem Tutulumu için Belirleyici Risk Faktörleri

Yıl 2021, Cilt: 54 Sayı: 3, 377 - 381, 01.01.2022

Tuba Kurt Zahide Ekici Tekin Elif Çelikel Fatma Aydın Müge Sezer Nilüfer Tekgöz Cüneyt Karagöl Serkan Coşkun Melike Kaplan Banu Acar

https://doi.org/10.20492/aeahtd.922021

Öz

AMAÇ: İmmünglobulin A vasküliti (IgAV), çocukluk döneminin en sık görülen küçük damar vaskülitidir. Alt ekstremitelerde görülen palpabl purpura karakteristik özelliğidir. Aynı zamanda eklem ağrısı, artrit, gastrointestinal sistem (GİS) ve üriner sistem etkilenmesi oldukça sıktır. Özellikle hastaneye yatışın büyük kısmı GİS tutulumu nedeni ile olmaktadır. Bu çalışmada IgAV’nde GİS tutulumu için risk faktörleri belirlenmeye çalışıldı.
GEREÇ ve YÖNTEM: Eylül 2015-Ağustos 2020 yılları arasında hastanemizin çocuk romatoloji kliniğinde IgAV tanısı ile izlenen GİS tutulumu olan ve olmayan toplam 100 hastanın dosyası retrospektif olarak incelendi. Klinik, demografik, laboratuar parametreleri ve uygulanan tedaviler kaydedildi.
BULGULAR: 100 (49 K/51 E) hastanın yarısı GİS tutulumu olan ve diğer yarısı GİS tutulumu olmayan hastalardan seçildi. Ortanca tanı yaşı 8 (6,1-9,8) yıldı. Tüm hastalarda palpabl purpura mevcuttu. En sık görülen diğer bulgular artrit (%34) ve artralji (%52) idi. GİS tutulumu olan hastaların tamamında karın ağrısı mevcuttu. Aktif kanama GİS tutulumu olan hastaların %25’inde (n=12) vardı. GİS tutulumu olan ve olmayan hastalar arasında yaş, cinsiyet, diğer klinik bulgular, mevsimsel farklılıklar, tanı anındaki laboratuar parametreleri arasında herhangi bir ilişki saptanmadı. Ancak MEFV gen mutasyonu varlığı, ağır GİS tutulumu olan grupta daha sık tespit edildi (p=0,009). Renal tutulum hastaların %18’inde gelişti. Hastaların tamamı komplikasyonsuz iyileşti.
SONUÇ: IgAV’nde GİS tutulumu oldukça sıktır. Bu çalışmada GİS tutulumunda yaş, cinsiyet, diğer klinik bulguların dağılımı, mevsimler gibi farklılıkların GİS tutulumu için belirleyici faktör olmadığını gösterdik. Ancak ağır GİS tutulumu olan hastalarda MEFV gen mutasyonlarının katkısı olabileceği göz önünde bulundurulmalıdır.
Abstract
AİM: Immunoglobulin A vasculitis (IgAV) is the most common small vessel vasculitis in childhood. It is characteristic of palpable purpura seen in the lower extremities. Also, joint pain, arthritis, gastrointestinal system (GI) and urinary system involvement are quite common. Especially most hospitalizations are due to GI involvement. In this study, it was aimed to determine the risk factors for GI involvement in IgAV.
MATERIALS AND METHODS: The files of 100 patients with and without GI involvement who were followed up with a diagnosis of IgAV in the pediatric rheumatology department of our hospital between September 2015 and August 2020 were retrospectively analyzed. Clinical, demographic, laboratory parameters and applied treatments were recorded.
RESULTS: Half of the 100 (49 F/51 M) patients were selected from patients with GI involvement and the other half from patients without GI involvement. The median age of diagnosis was 8 (6.1-9.8) years. All patients had palpable purpura. The other most common findings were arthritis (34%) and arthralgia (52%). All patients with GI involvement had abdominal pain. Active bleeding was present in 25% (n = 12) of the patients with GI involvement. No relationship was found between patients with and without GIS involvement, between age, gender, other clinical findings, seasonal differences, and laboratory parameters at the time of diagnosis. However, presence of MEFV gene mutation was detected more frequently in the group with severe GI involvement (p = 0.009). Renal involvement developed in 18% of the patients. All the patients recovered without any complications.
CONCLUSION: GI involvement is very common in IgAV. In this study, we showed that differences in GI involvement such as age, gender, distribution of other clinical findings, and seasons are not determinative factors for GI involvement. However, it should be kept in mind that MEFV gene mutations may contribute to patients with severe GI involvement.
Key words: IgA vasculitis, gastrointestinal system, risk, MEFV gene

Anahtar Kelimeler

IgA vasküliti, gastrointestinal sistem, risk, MEFV

Kaynakça

• 1.) Trnka P. Henoch–schönlein purpura in children. J Paediatr Child Health. 2013;49(12):995–1003.
• 2.) Roache-Robinson P, Hotwagner DT. Henoch Schönlein Purpura. In: StatPearls. Treasure Island (FL): StatPearls Publishing; December 2, 2020.
• 3.) Hetland LE, Susrud KS, Lindahl KH, et al. Henoch-Schönlein Purpura: A Literature Review. Acta Derm Venereol. 2017 Nov 15;97(10):1160-1166.
• 4.) Oni L, Sampath S. Childhood IgA Vasculitis (Henoch Schonlein Purpura)-Advances and Knowledge Gaps. Front Pediatr. 2019; 7:257.
• 5.) Tabel Y, Inanc FC, Dogan DG et al. Clinical features of children with Henoch- Schonlein purpura: risk factors associated with renal involvement. Iranian journal of kidney diseases. 2012; 6: 269-74.
• 6.) K. Kawasaki, H. Komura, Y. Nakahara, et al., Factor XIII in Henoch- Schönlein purpura with isolated gastrointestinal symptoms, Pediatr. Int. 48 (2006) 413–415.
• 7.) Yakut HI, Kurt T, Uncu N, et al. Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement. Arch Argent Pediatr. 2020;118(2):139-142.
• 8.) Shi D, Chan H, Yang X, et al. (2019) Risk factors associated with IgA vasculitis with nephritis (Henoch–Schönlein purpura nephritis) progressing to unfavorable outcomes: A meta-analysis. PLoS ONE 14(10): e0223218.
• 9.) Ozen S, Pistorio A, Iusan SM, et al; Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010; 69:798–806
• 10.) Yalcinkaya F, Ozen S, Ozcakar ZB, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford, England). 2009; 48(4):395–398.
• 11.) Reamy BV, Servey JT, Williams PM. Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review. Am Fam Physician. 2020 Aug 15;102(4):229-233.
• 12.) Ebert EC. Gastrointestinal manifestations of Henoch- Schonlein Purpura. Dig Dis Sci. 2008; 53(8):2011–2019.
• 13.) Ekinci RMK, Balci S, Sarı GS, et al. Do practical laboratory indices predict the outcomes of children with Henoch-Schönlein purpura? Postgrad Med. 2019 May;131(4):295-298.
• 14.) Hwang HH, Lim IS, Choi BS, et al. Analysis of seasonal tendencies in pediatric Henoch-Schonlein purpura and comparison with outbreak of infectious diseases. Medicine (Baltimore). 2018; 97(36): e12217
• 15.) Karadağ ŞG, Çakmak F, Çil B, et al. The relevance of practical laboratory markers in predicting gastrointestinal and renal involvement in children with Henoch-Schönlein Purpura. Postgrad Med. 2020 Aug 19:1-6.
• 16.) Chen O, Zhu XB, Ren P, et al. Henoch Schonlein Purpura in children: clinical analysis of 120 cases. African Health Sciences. 2013; 13:94-9.
• 17.) Ben-Chetrit E, Touitou I. FamilialMediterranean fever in the world. Arthritis Rheum. 2009; 61:1447–1453.
• 18.) Lachmann HJ, Sengul B, Yavuzsen TU et al. Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford). 2006; 45:746–750.
• 19.) Can E, Kılınç YZ, Hamilçıkan S, et al. MEFV gene mutations and clinical course in pediatric patients with Henoch-Schönlein purpura. Arch Argent Pediatr. 2018;116(3): e385-e391.
• 20.) Ozçakar ZB, Yalçinkaya F, Cakar N, et al. MEFV mutations modify the clinical presentation of Henoch-Schönlein purpura. J Rheumatol. 2008; 35(12):2427–2429.
• 21.) Cakici EK, Kurt Şükür ED, Özlü SG, et al. MEFV gene mutations in children with Henoch-Schönlein purpura and their correlations-do mutations matter? Clin Rheumatol. 2019 Jul;38(7):1947-1952.
• 22.) Gershoni-Baruch R, Broza Y, Brik R. Prevalence and significance of mutations in familial Mediterranean fever gene in Henoch- Schonlein purpura. J Pediatr. 2003;143(5):658–661.