Nazan ŞEN, Eftal YÜCEL, Reyhan YILDIZ, Müge AYDIN TUFAN, Emine Duygu ERSÖZLÜ

Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up

Granülomatöz polianjit (Wegener granülomatozis): Klinik bulgular ve uzun dönem izlem sonuçları Giriş: Üçüncü basamak bir üniversite hastanesinde granülomatöz polianjit (GPA) tanılı hastaların uzun dönem izlem sonuçlarının değerlendirilmesi.Materyal ve Metod: Hastanemizde 2004-2014 tarihleri arasında, tanısı doku biyopsileri ile doğrulanmış 22 GPA'lı hastanın medikal kayıtları incelendi. Bulgular: Semptomların başlangıcından tanıya kadar geçen süre 7.8 ± 12.3 ay [interquartile range (IR)= 4.0] olarak bulundu. En sık tutulan organlar üst solunum yolu (ÜSY) (%72.7), alt solunum yolu (%81.8) ve böbreklerdi (%72.7). ÜSY tutulumu iyi prognoz göstergesiydi (p= 0.046). Sağkalım süresi ÜSY tutulumu olan ve olmayan hastalarda sırasıyla 124.6 ± 6.9 ay ve 59.7 ± 22.9 aydı. Diyaliz gerektiren son dönem böbrek yetmezliği (SDBY) ve kardiyak tutulum mortalite ile ilişkili bulundu (sırasıyla p= 0.022 ve p= 0.026). Tanı sırasında diyalize bağımlı 12 hastanın 11'i 3 aydan uzun süre yaşadı ve bunların 7'sinde böbrek fonksiyonları kalıcı olarak düzeldi. Plazmaferez uygulanan hastalarda diyaliz bağımlılığı anlamlı olarak düşüktü (p= 0.047). Toplam mortalite oranı %18 (4/22) olarak bulundu. Ortalama sağkalım 55.9 ± 42.8 aydı (IR= 84.0).Sonuç: Hastalığın nonspesifik semptomları nedeniyle GPA tanısında gecikmeler yaşanabilmektedir. Çalışmamızda ÜSY tutulumu iyi prognoz göstergesi olarak bulunurken, kardiyak tutulum ve diyaliz gerektiren SDBY kötü prognoz ile ilişkili bulundu. Plazmaferez diyaliz gerektiren SDBY'li hastalarda iyileşme hızını artırabilir.

Granülomatöz polianjit (Wegener granülomatozis): Klinik bulgular ve uzun dönem izlem sonuçları

Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Results: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 ± 6.9 months and 59.7 ± 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 ± 42.8 months (IR= 84.0).Conclusion: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.

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