Demographics, clinical, laboratory findings and treatment results of pediatric patients with IgA Vasculitis: Single-center experience

Abstract

Objective: Immunoglobulin A (IgA) vasculitis (IgAV), also known as Henoch-Schönlein purpura (HSP), is a vasculitis characterized by the accumulation of IgA in the vessel walls. In this study, we purposed to evaluate the demographics, clinical and laboratory findings, and treatments and responses of patients diagnosed with IgAV/HSP in our center.
Materials and methods: The records of 201 IgAV/HSP patients who were followed up in the pediatric nephrology-rheumatology clinic were evaluated retrospectively.
Results: It was seen with the equal frequency between girls and boys. While all patients had purpura, other findings were gastrointestinal, joint, renal, subcutaneous edema, and testicular involvement, in order of frequency. The rate of patients who developed intussusception was 2.5%, and none required surgical treatment. Biopsy was performed in patients with persistent proteinuria or hematuria. Histopathological diagnoses were mesangial proliferation, crescent, and minimal change, respectively. While the rate of renal involvement was high in cases with rash and relapse (p=0.046), there was no difference in gastrointestinal and joint involvement. In the histopathological findings of the boys, the crescent was higher than in the girls (p=0.017).
Conclusion: IgAV/HSP generally has a good prognosis, but some patients suffer from renal involvement. In our study, renal histopathology in cases with renal involvement showed milder findings in girls than in boys, but there was no difference in other findings. Renal involvement was higher in relapsed patients.

Keywords

• IgA vasculitis
• renal involvement
• Henoch-Schonlein purpura
• renal histopathology
• IgA vasküliti
• renal tutulum
• Henoch-Schonlein purpurası
• renal histopatoloji

IgA Vaskülitli çocuk hastaların demografik, klinik, laboratuvar bulguları ve tedavi sonuçları: tek merkez deneyimi

Öz

Amaç: Henoch-Schönlein purpurası (HSP) olarak adlandırılan İmmünoglobin A (IgA) vasküliti (IgAV), damar duvarlarında IgA baskın immün birikimi ile karakterize küçük damar vaskülitidir. Bu çalışmada merkezimizde IgAV/HSP tanısı almış hastaların demografik özelliklerini, klinik ve laboratuvar bulgularını tedavilerini ve tedavi yanıtlarını değerlendirmeyi amaçladık.
Gereç ve yöntem: Çocuk romatoloji ve nefroloji kliniğinde takipli 201 IgAV/HSP hastasının kayıtları retrospektif olarak değerlendirildi.
Bulgular: Kız ve erkekler arasında hastalık eşit sıklıkta görüldü. Hastaların tamamında purpura varken diğer bulgular sıklık sırasına göre gastrointestinal, eklem, renal, subkutan ödem, testiküler tutulum idi. İnvajinasyon gelişen hastaların oranı %2,5’tu ve hiçbirinde cerrahi tedavi gerekmedi. Persistan proteinüri veya hematürisi olan hastalara biyopsi uygulandı. Histopatolojik tanıları sırayla mezengial proliferasyon, kresent ve minimal değişiklik idi. Döküntü ile relaps gelişen olgularda renal tutulum oranı yüksek iken (p=0,046) gastrointestinal ve eklem tutulum oranlarında fark yoktu. Renal tutulum olan erkeklerin böbrek histopatolojik bulgularında kresent kızlara göre daha yüksek oranda görüldü (p=0,017).
Sonuç: IgAV/HSP, genel olarak, iyi prognoza sahiptir, ancak renal tutulumdan muzdarip hastalar vardır. Çalışmamızda renal tutulum gerçekleşen olgularda böbrek histopatolojisi kızlarda erkeklere göre daha hafif bulgular gösterirken diğer bulgularda fark yoktu. Relaps gelişen olgularda renal tutulum daha fazla idi.

Anahtar Kelimeler

• IgA vasküliti
• renal tutulum
• Henoch-Schonlein purpurası
• renal histopatoloji

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