Nefrotik Düzey Proteinüri ile Başvurunun Çocukluk Çağı Iga Vasküliti Nefriti Seyrine Etkileri

Öz

İmmünglobulin A vasküliti (IgAV) palpabl purpura’ya ek olarak karın ağrısı, artrit, hematüri ve/veya proteinüri, IgA baskın depolanma gösteren doku biyopsisi kriterlerinden en az birisinin birlikteliği ile tanısı konulan bir küçük damar vaskülitidir. Bu hastalarda %30-50 oranlarında değişken prezentasyonlarla böbrek tutulumu görülebilmektedir. IgAV nefritinde (IgAVN) proteinüri renal prognoz hakkında fikir vericidir. Bu çalışmada çocukluk çağı IgAVN’de proteinüri miktarının klinikopatolojik özellikler ve renal sağkalım ile ilişkisini değerlendirmek amaçlanmıştır. Çalışmaya Ocak 2005-Aralık 2021 tarihleri arasında biyopsi ile IgAVN tanısı kesinleştirilmiş 48 çocuk hasta dahil edildi. Hastaların başvuru, 3.ay, 6.ay, 12. ay ve son başvuru dönemlerine ait klinik, laboratuvar ve patolojik bulgular, aldıkları tedaviler kaydedildi. Başvuru proteinüri miktarlarına göre hastalar nefrotik düzeyde olan ve olmayan olarak iki gruba ayrıldı. Renal sağkalım için Meadow sınıflaması kullanıldı. Hastaların ortanca tanı yaşı 8 (6-12), erkek/kız oranı 1.1 idi. Nefrotik düzey proteinürisi olan 34 hasta mevcuttu, bu hastalarda IgAV tanısından böbrek tutulumuna ve renal semptom başlangıcından biyopsi yapılmasına kadar geçen süre daha kısa (p=0.027), başvuru eGFR değerleri daha yüksekti (p=0.021). Nefrotik düzey proteinürisi olan hastaların biyopsilerinde kresent yüzdesi (p=0.036), klinikte steroide ek immunsupresif kullanımı daha fazla (p=0.011), remisyona girme süresi anlamlı olarak daha uzundu (p=0.016) ancak proteinürisi nefrotik düzeyde olan ve olmayanların renal sağkalım oranlarında anlamlı fark bulunmadı (p=0.469). Sonuç olarak, IgAVN’de proteinüri önemli bir bulgudur ve proteinürinin miktarı arttıkça böbrek hasarına katkısı artar. Daha ağır biyopsi bulguları, yoğun immunosupresif tedavi ihtiyacı ve uzamış remisyona girme süreleri göz önüne alındığında IgAV hastalarında düzenli aralıklarla idrarda protein taraması yapmak, erken tedavi başlanarak olası komplikasyonların önüne geçmek açısından önemlidir.

Anahtar Kelimeler

• çocuk
• immunglobulin A vasküliti
• proteinüri
• prognoz

Effects of Presentation with Nephrotic Range Proteinuria on Childhood Iga Vasculitis Nephritis

Öz

Immunoglobulin A vasculitis (IgAV) is a small vessel vasculitis diagnosed by palpable purpura with at least one of following criteria; abdominal pain, arthritis, hematuria and/or proteinuria,IgA-predominant deposition in tissue samples. Renal involvement can be seen in 30-50% of the patients. Proteinuria in IgAV nephritis (IgAVN) is predictive of renal prognosis. In this study, we aimed to evaluate the relationship between the degree of proteinuria and clinicopathological features, renal survival in childhood IgAVN. Forty-eight patients with biopsy confirmed IgAVN between January 2005 and December 2021 were included in the study. Clinical, laboratory and pathological findings at admission, 3rd, 6th, 12th months and last visit, treatments they received were recorded. Based on degree of proteinuria at admission, patients were divided into two groups as nephrotic and non-nephrotic. Meadow classification was used for renal survival. The median age of the patients was 8 (6-12) years, and male/female ratio 1.1. There were 34 patients with nephrotic range proteinuria. In the nephrotic group, the time from IgAV to IgAVN and from IgAVN to biopsy was shorter (p=0.027), and eGFR values at admission were higher (p=0.021). In the these patients amount of crescents in biopsy (p=0.036), immunosuppressive use were more common (p=0.011) and time to remission was longer (p=0.016). No significant difference in renal survival was observed among both groups (p=0.469). In conclusion, proteinuria is an important finding in IgAVN. Considering aggressive biopsy findings, need for intensive immunosuppressive therapy, and delayed remission times, IgAV patients should be screened for proteinuria to prevent possible complications.

Anahtar Kelimeler

• child
• immunoglobulin A vasculitis
• proteinuria
• prognosis

Kaynakça

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