Clinical Features and Outcomes of Patients with Granulomatosis with Polyangiitis and Review of Literature

Objective: Granulomatosis with polyangiitis (GPA) is a type of antineutrophil cytoplasm antibody-associated vasculitis. This study aimed to investigate the clinical and laboratory findings of patients with GPA and to determine which factors were associated with poor prognosis and renal outcome.

Materials and Methods: Medical records of patients diagnosed with GPA between 2000 and 2014 were retrospectively analyzed.

Results: A total of 53 patients (60.4% men) with a mean age at diagnosis of 47.1±16.3 years were included in this study. The median baseline serum creatinine (SCr) level was 1 (interquartile range [IQR], 0.76-2.95) mg/dL. Renal involvement was detected in 63.5% of the patients. Patients with renal involvement had less ear-nose-throat involvement (p=0.002) and lower hematocrit levels (p=0.001). The 1-year, 5-year, and 10-year death-censored renal survival rates were 93.3%, 81.4%, and 63.4%, respectively. There were 15 deaths (28.3%) in this series, and the median patient survival after diagnosis was 51 (IQR, 20-109.5) months. Age ≥65 years and SCr level ≥1.4 mg/dL at diagnosis were retained as the independent predictors of mortality.

Conclusion: Survival of patients with GPA is closely associated with age and renal function at admission. The delay in diagnosis could have influenced the development of irreversible kidney damage, one of the significant determinants of mortality.

Cite this article as: Arslan E, Oruç M, Yalın SF, Alagöz S, Demir H, Hatemi G, et al. Clinical Features and Outcomes of Patients with Granulomatosis with Polyangiitis and Review of Literature. Turk J Nephrol 2020; 29(4): 279-88.