title: Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up.
abstract: BACKGROUND
  To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital.
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Chest Diseases, Baskent University Adana Dr. Turgut Noyan Teaching and Medical Research Center, Adana, Turkey. nazansen68@gmail.com.
authors.forename: Nazan
authors.initials: N
authors.lastname: Şen
authors.forename: Müge
authors.initials: M
authors.lastname: Aydın Tufan
authors.forename: Reyhan
authors.initials: R
authors.lastname: Yıldız
authors.forename: Emine Duygu
authors.initials: ED
authors.lastname: Ersözlü Bozkırlı
authors.forename: Eftal
authors.initials: E
authors.lastname: Yücel
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies