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 ...

Extracorporeal membrane oxygenation (ECMO) was developed as a supportive therapy to treat severe respiratory failure. When conventional mechanical ventilation has failed or when there is not enough time to treat the pathology, ECMO has the potential to sustain life. In this report, successful use of ECMO to support an adult patient with antineutrophil cytoplasmic antibody (ANCA)-associated syst...

INTRODUCTION S hunt nephritis is a rare complication mostly described in the setting of chronic infection of ventriculoatrial (VA) shunts inserted for the treatment of congenital or acquired hydrocephalus. The diagnosis of shunt nephritis is challenging and may be overlooked. We report a case of successfully treated shunt nephritis and pyogenic spondylitis with positive antiproteinase 3 antineu...

OBJECTIVE To investigate the diagnosis and treatment of mixed connective tissue disease (MCTD) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis, which is a rare clinical entity in medical practice. METHODS A 35-year-old female of Asian origin was admitted to our hospital due to complaints of Raynaud's phenomenon, myalgia, arthralgia and fatigue. The pat...

1 Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 1999;78:26–37. 2 Val-Bernal JF, Mayorga M, Garcia-Alberdi E, Pozueta JA. Churg-Strauss syndrome and sudden cardiac death. Cardiovasc Pathol 2003;12:94–7. 3 Sable-Fourtassou R, Cohen P, Mahr A et al. Antineutrophil cytoplasmic antibodies and...

The antineutrophil cytoplasmic antibody (ANCA) vasculitides include several closely related, often severe, multisystem autoimmune diseases characterized by antibodies against serine proteinase 3 (PR3) or myeloperoxidase. Loss of tolerance to these antigens triggers a cascade events, beginning with the priming neutrophils proinflammatory cytokines and complement activation, translocation ANCA-sp...

IgG4 disease was first described in 2001 in a seminal paper by Hamano and colleagues: Syndrome of exocrine gland infiltration with associated raised serum IgG4 levels. IgG4 disease is manifested by the hallmark histopathologic features of lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. Hypertrophic pachymeningitis (HP) has been incre...

INTRODUCTION Antineutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (GN) is characterized by necrotizing and crescentic GN with paucity of immunoglobulin (Ig) and complement deposition, which is also known as pauci-immune crescentic GN. Membranous nephropathy (MN) is characterized by the formation of subepithelial immune deposit with resultant changes in glomerular basement ...

OBJECTIVE To study the sera from selected groups of antineutrophil cytoplasmic antibody (ANCA) positive patients by means of the indirect immunofluorescence test (ANCA-IIF) with different fixatives, in order to better discriminate among the various ANCAs (Ag-specificity and disease associations), especially those for which the antigen targets have not yet been identified. METHODS Eighty patho...