Clinicopathological analysis of patients with granulomatosis with polyangiitis (Wegener's) complicated by acute renal failure

Abstract

To analyze the clinicopathological features of patients with granulomatosis with polyangiitis (Wegener's) (GPA) complicated by acute renal failure (ARF). A total of 27 GPA patients with ARF at our hospital from January 1988 to December 2011 were recruited into this retrospective study. A structured interview, systemic rheumatologic examination, laboratory tests (including autoantibodies) and renal pathology were conducted. And their treatment and prognosis were also analyzed. The incidence of GPA with ARF was 15% and the average age 50 years old. The common symptoms were fever (n = 23) and weight loss (n = 15) while the most common extra-renal organ damages affected lung (n = 20) and nose (n = 19). Renal damages manifested as mild or moderate proteinuria (n = 26) and hematuria (n = 25). However, oliguria and anuria were rare. Anti-neutrophil cytoplasma antibodies (ANCA) were positive in 23 patients. Among them, 22 were identified by proteinase 3. Renal biopsies revealed pauci-immune crescentic glomerulonephritis. Combined treatment of glucocorticoid and immunosuppressants was effective. Twenty-two patients became relieved and only 4 patients died. As a common complication of GPA, ARF has a rapid onset and a fast progression. Oliguria and anuria are rare. Renal biopsy is not only vital in the diagnosis and evaluation of disease activity, but also helpful for therapeutic decision-making.