A Case of Microscopic Polyangiitis: Glomerulonephritis, Neuropsychiatric Abnormalities, and Urticarial Vasculitis / 대한신장학회지

ABSTRACT

We report a case of microscopic polyangiitis, presenting with rapidly progressive glomerulonephritis, neuropsychiatric abnormalities, and urticarial vasculitis. A 65-year-old woman reported loss of appetite, significant weight loss, and a transient history of veering tendency. She was presented with a mild fever, cough, and sputum. Routine laboratory test revealed anemia, leukocytosis with a left shift, azotemia, and elevated highly sensitive C-reactive protein. The bilateral kidneys were observed to be enlarged (right kidney 16.3 cm, left kidney 18.2 cm) on an abdominal computed tomography. The perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) titer was >1640 and MPO-ANCA was positive. Her chief complaints consisted of character change, visual illusion, and hearing loss. A skin rash with a bullous change resembling urticaria was further developed. Kidney biopsy demonstrated pauci-immune diffuse crescentic glomerulonephritis. Skin biopsy was compatible with urticarial vasculitis. Having received intravenous cyclophosphamide and oral prednisolone, she showed sustained improvement in renal function, as well as her neuropsychiatric symptoms and skin rash.