Hilar and para-aortic necrotizing lymphadenopathy associated with antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis

Lymph node involvement as a part of systemic vasculitis is rare. We report a case of women with rapidly progressive renal disease associated with recurrent epigastric pain, weight loss, and massive hilar as well as para-aortic lymphadenopathy. Ultrasound-guided biopsy of her scarred kidneys revealed antineutrophil cytoplasmic antibody-negative crescentic glomerulonephritis and that of lymph nodes showed severe necrotizing vasculitis. Biopsy of the lymph nodes and the failing kidney established the diagnosis of this rare presentation and ruled out lymphoma and tuberculosis. Administration of corticosteroids and cyclophosphamide resulted in a favorable outcome.