ANCA-associated large and small vessel vasculitis with aorto-pulmonary-renal syndrome

A 65-year-old hypertensive man presented to the Government Medical College Associated Superspeciality Hospital (Srinagar, India) with a 3-month history of progressive fatigue, anorexia, weight loss, and dull abdominal pain. On evaluation, he was found to have hypochromic, microcytic anaemia, and renal dysfunction (blood urea of 147 mg/dL and serum creatinine of 4·2 mg/dL). He had microscopic haematuria with dysmorphic red blood cells, 3+ proteinuria on dipstick analysis (300 mg/dL), and a 24-h urinary protein concentration of 1300 mg. His upper gastrointestinal endoscopy and colonoscopy were normal. A high-resolution CT of the man's chest showed a diffuse ground glass appearance. A CT angiography of his abdomen revealed diffuse aortitis of the abdominal aorta and both iliac arteries, without any luminal narrowing (figure A–C) or radiological evidence of extension of the inflammatory process into the periaortic space. A CT angiography of the man's thorax did not reveal any involvement of the thoracic aorta or subclavian arteries. A Doppler ultrasound of his temporal arteries was normal. Other blood tests showed an erythrocyte sedimentation rate of 80 mm/h and a C-reactive protein concentration of 111 mg/L. Hepatitis B, C, and HIV serologies were negative. Tests for antinuclear and antiglomerular basement membrane antibodies were negative. An antimyeloperoxidase antineutrophil cytoplasmic antibody (ANCA) ELISA was positive (121 U/mL [typical<5 U/mL]). Over the next few days, the man developed haemoptysis and a worsening estimated glomerular filtration rate (GFR) requiring several sessions of dialysis. A kidney biopsy was done, which revealed pauci-immune necrotising crescentic glomerulonephritis (figure D). A diagnosis of ANCA-associated large vessel vasculitis of the abdominal aorta and iliacae communis, and small vessel vasculitis with necrotising crescentic glomerulonephritis, and pulmonary capillaritis, causing an aorto-pulmonary-renal syndrome, was made. This was managed with intravenous methylprednisone followed by oral prednisone and intravenous cyclophosphamide, which led to an improvement in his symptoms and estimated GFR. The man died of a massive pulmonary haemorrhage at home, a few weeks post discharge, after having discontinued his immunosuppressants against medical advice.