Çocukluk Çağında İmmunglobulin A vasküliti: Tek Merkez Deneyimi

Year 2021, Volume: 43 Issue: 5, 512 - 518, 13.09.2021

Elif Çelikel , Ulaş Özdemir

https://doi.org/10.20515/otd.944463

Abstract

Öz:
Amaç: Bu çalışmada, immunglobulin A (IgA) vasküliti tanısı ile takip edilen çocukların klinik özelliklerinin, laboratuvar bulgularının, tedavilerinin ve prognozunun değerlendirmesi amaçlanmıştır.
Gereç ve Yöntem: 2018-2020 tarihleri arasında merkezimiz Çocuk Romatoloji Kliniği'nde IgA vasküliti tanısı alan, en az 6 ay izlemine devam edilen, 139 çocuğun tıbbi kayıtları geriye dönük olarak değerlendirildi.
Sonuç: 139 hastanın 75’i kızdı (%53,9). Kız/erkek oranı 1,17 olarak saptandı. Hastaların ortalama yaşı 7,6±2,9 yıl olarak tespit edildi. Hastalığın başlamasından önce hastaların 118’inde (%84) geçirilmiş üst solunum yolu enfeksiyon öyküsü mevcuttu. Klinik bulgulardan döküntü, hastaların tamamında mevcuttu. Hastaların 91’inde (%65,5) cilt altı ödem, 89’unda (%64) artralji; 68’inde (%48,9) artrit, 54’ünde (%38,8) karın ağrısı, 30’unda (%21,5) renal tutulum, 6’sında (%4.3) testis tutulumu tespit edildi. Laboratuar bulguları değerlendirildiğinde hastaların 28’inde (% 20,1) lökositoz ve trombositoz, 29’unda (%20,8) akut faz reaktanlarında yükseklik tespit edildi. İzlemde 30 hastada renal tutulum gelişti. Renal tutulum olan 9 hasta (%30) tedavisiz izlenirken, 18’ine diğer sistem tutlumları nedeni ile nonsteroid antiinflamatuvar ilaç, 3’üne (%10) renal tutulum için steroid tedavisi verildi. Dört hasta (1 makroskobik hematüri, 3 nefrotik proteinüri) çocuk nefroloji bölümüne yönlendirildi. Hastaların 30’u (%21,5) tedavisiz izlenirken 56 hastaya (%40,2) non-steroidal antiinflamatuar ilaç (NSAİİ), 51 hastaya (%36,6) steroid tedavisi verildi. Takipte 17 (%12,2) hastada hastalık tekrarladı.
Sonuç: IgA vasküliti cilt ve eklemler başta olmak üzere birçok organ ve sistemi tutabilen bir vaskülittir, hastaların çoğu sekelsiz iyileşmektedir. Bununla beraber erken ve geç dönemde gelişebilecek gastrointestinal ve böbrek tutulumu gibi komplikasyonlar açısından hastaların yakın takip edilmesi gerekmektedir.

Keywords

IgA vasküliti, Çocukluk çağı, MEFV, prognoz

Supporting Institution

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Immunoglobulin A Vasculitis in Childhood: One-Center Experience

Abstract

The aim of this study was to evaluate the clinical characteristics, laboratory findings, treatment and prognosis of children with immunoglobulin A (IgA) vasculitis diagnosis. The medical records of 139 children who were diagnosed with IgA vasculitis in our center's Pediatric Rheumatology Clinic between 2018-2020, who continued follow-up for at least 6 months, were retrospectively evaluated. 75 of 139 patients were girls (53.9%). The male / female ratio was 1.17. The average age of the patients was 7.6±2.9 years. Prior to the onset of the disease, 118 (84%) of patients had a history of upper respiratory tract infection. From clinical findings, the rash was present in all patients. Subcutaneous edema in 91 (65.5%), arthralgia in 89 (64%); arthritis was detected in 68 (48.9%), abdominal pain in 54 (38.8%), renal involvement in 30 (21.5%), testicular involvement in 6 (4.3%) of the patients. In the laboratory evaluation, leukocytosis and thrombocytosis were detected in 28 (20.1%), and increased in acute phase reactants in 29 (20.8%) of the patients. During the follow-up, renal involvement developed in 30 patients. Nine patients (30%) with renal involvement were followed without treatment, 18 were given nonsteroidal anti-inflammatory drugs for other system involvement, and steroid treatment was given to 3 (10%) for renal involvement. Four patients (1 macroscopic hematuria, 3 nephrotic proteinuria) were referred to the pediatric nephrology department. Thirty (21.5%) of the patients were followed without treatment, 56 patients (40.2%) were given non-steroidal anti-inflammatory drugs, and 51 (36.6%) patients were treated with steroids. The disease recurred in 17 (12.2%) patients during follow-up. IgA vasculitis is a vasculitis that can involve many organs and systems, especially the skin and joints, and most of the patients recover without sequelae. However, patients should be followed up closely in terms of complications such as gastrointestinal and kidney involvement that may develop in the early and late stages.

Keywords

IgA vasculitis, childhood, MEFV, prognosis

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