Pulmonary renal syndrome is a rare but serious complication of systemic vasculitis. The majority the cases are antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. In this case, a 58 years old female case having aworsening clinical picture of probable urinary infection and acute renal failure was presented. By means of proteinuria, pyuria, hematuria and unexplained acute renal failure, we review the relevant literature on pulmonary renal syndrome associated with granulomatosis with polyangiitis (GPA) with negative serum ANCA serology. This report demonstrates the difficulty of diagnosing granulomatosis with polyangiitis (GPA) until renal biopsy was done while initial diagnostic serological negative ANCA testing. [Int J Res Med Sci 2016; 4(7.000): 3039-3041
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