title: Marfan syndrome with antineutrophil cytoplasmic antibody-associated systemic vasculitis presenting as severe anaemia and haematuria after the Bentall procedure.
abstract: One month previously, a 28-year old male underwent an emergency modified Bentall procedure because of Marfan syndrome with acute aortic dissection Stanford Class A. Computed tomography of the chest did not reveal severe graft stenosis of the anastomosis. To explore the cause of anaemia, renal dysfunction and macroscopic haematuria, the patient was tested for antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV). Antimyeloperoxidase antibodies (MPO)-ANCA and antiproteinase 3 antibodies (PR3)-ANCA were strongly positive. Corticosteroid therapy was applied, followed by cyclophosphamide and azathioprine. In response to treatment, the MPO-ANCA and PR3-ANCA levels gradually decreased, proteinuria was alleviated and haemoglobin levels returned to normal after 6 months. This is the first report to highlight haemolytic anaemia and AASV with Marfan syndrome after surgery for aortic dissection.
authors: Department of Nephrology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors: Li
authors: L
authors: Sijia
authors: Liu
authors: L
authors: Shuangxin
authors: Shi
authors: S
authors: Wei
authors: Cui
authors: C
authors: Yanhai
mesh_terms: D000328:Adult; D000740:Anemia / Q000175:diagnosis*; D000784:Aortic Dissection / Q000601:surgery; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D001014:Aortic Aneurysm / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000379:methods*; D006417:Hematuria / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000097:blood / Q000175:diagnosis* / Q000601:surgery*; D056647:Systemic Vasculitis / Q000097:blood / Q000175:diagnosis*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Anaemia; Antineutrophil cytoplasmic antibody-associated systemic vasculitis; Haematuria; Marfan syndrome