학술논문
Demographics, clinical, laboratory findings and treatment results of pediatric patients with IgA Vasculitis: single-center experiences/ IgA Vaskulitli cocuk hastalarin demografik, klinik, laboratuvar bulgulari ve tedavi sonuclari: tek merkez deneyimi
Research Article
Research Article
Document Type
Clinical report
Source
Pamukkale Medical Journal. January 2023, Vol. 16 Issue 1, p73, 8 p.
Subject
Language
English
ISSN
1309-9833
Abstract
Introduction Immunoglobin A (IgA) vasculitis (IgAV), previously named Henoch-Schonlein purpura (HSP), is a small vessel vasculitis characterized by IgA-dominant immune accumulation in the vessel walls [1]. While it may occur [...]
Purpose: Immunoglobulin A (IgA) vasculitis (IgAV), also known as Henoch-Schonlein 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. Key words: IgA vasculitis, renal involvement, Henoch-Schonlein purpura, renal histopathology. Amac: Henoch-Schonlein purpurasi (HSP) olarak adlandirilan Immunoglobin A (IgA) vaskuliti (IgAV), damar duvarlarinda IgA baskin immun birikimi ile karakterize kucuk damar vaskulitidir. Bu calismada merkezimizde IgAV/HSP tanisi almis hastalarin demografik ozelliklerini, klinik ve laboratuvar bulgularini tedavilerini ve tedavi yanitlarini degerlendirmeyi amacladik. Gerec ve yontem: Cocuk romatoloji ve nefroloji kliniginde takipli 201 IgAV/HSP hastasinin kayitlari retrospektif olarak degerlendirildi. Bulgular: Kiz ve erkekler arasinda hastalik esit siklikta goruldu. Hastalarin tamaminda purpura varken diger bulgular siklik sirasina gore gastrointestinal, eklem, renal, subkutan odem, testikuler tutulum idi. Invajinasyon gelisen hastalarin orani %2,5'tu ve hicbirinde cerrahi tedavi gerekmedi. Persistan proteinuri veya hematurisi olan hastalara biyopsi uygulandi. Histopatolojik tanilari sirayla mezengial proliferasyon, kresent ve minimal degisiklik idi. Dokuntu ile relaps gelisen olgularda renal tutulum orani yuksek iken (p=0,046) gastrointestinal ve eklem tutulum oranlarinda fark yoktu. Renal tutulum olan erkeklerin bobrek histopatolojik bulgularinda kresent kizlara gore daha yuksek oranda goruldu (p=0,017). Sonuc: IgAV/HSP, genel olarak, iyi prognoza sahiptir, ancak renal tutulumdan muzdarip hastalar vardir. Calismamizda renal tutulum gerceklesen olgularda bobrek histopatolojisi kizlarda erkeklere gore daha hafif bulgular gosterirken diger bulgularda fark yoktu. Relaps gelisen olgularda renal tutulum daha fazla idi. Anahtar kelimeler: IgA vaskuliti, renal tutulum, Henoch-Schonlein purpurasi, renal histopatoloji.
Purpose: Immunoglobulin A (IgA) vasculitis (IgAV), also known as Henoch-Schonlein 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. Key words: IgA vasculitis, renal involvement, Henoch-Schonlein purpura, renal histopathology. Amac: Henoch-Schonlein purpurasi (HSP) olarak adlandirilan Immunoglobin A (IgA) vaskuliti (IgAV), damar duvarlarinda IgA baskin immun birikimi ile karakterize kucuk damar vaskulitidir. Bu calismada merkezimizde IgAV/HSP tanisi almis hastalarin demografik ozelliklerini, klinik ve laboratuvar bulgularini tedavilerini ve tedavi yanitlarini degerlendirmeyi amacladik. Gerec ve yontem: Cocuk romatoloji ve nefroloji kliniginde takipli 201 IgAV/HSP hastasinin kayitlari retrospektif olarak degerlendirildi. Bulgular: Kiz ve erkekler arasinda hastalik esit siklikta goruldu. Hastalarin tamaminda purpura varken diger bulgular siklik sirasina gore gastrointestinal, eklem, renal, subkutan odem, testikuler tutulum idi. Invajinasyon gelisen hastalarin orani %2,5'tu ve hicbirinde cerrahi tedavi gerekmedi. Persistan proteinuri veya hematurisi olan hastalara biyopsi uygulandi. Histopatolojik tanilari sirayla mezengial proliferasyon, kresent ve minimal degisiklik idi. Dokuntu ile relaps gelisen olgularda renal tutulum orani yuksek iken (p=0,046) gastrointestinal ve eklem tutulum oranlarinda fark yoktu. Renal tutulum olan erkeklerin bobrek histopatolojik bulgularinda kresent kizlara gore daha yuksek oranda goruldu (p=0,017). Sonuc: IgAV/HSP, genel olarak, iyi prognoza sahiptir, ancak renal tutulumdan muzdarip hastalar vardir. Calismamizda renal tutulum gerceklesen olgularda bobrek histopatolojisi kizlarda erkeklere gore daha hafif bulgular gosterirken diger bulgularda fark yoktu. Relaps gelisen olgularda renal tutulum daha fazla idi. Anahtar kelimeler: IgA vaskuliti, renal tutulum, Henoch-Schonlein purpurasi, renal histopatoloji.