title: Rapidly progressive lupus nephritis with extremely high levels of antineutrophil cytoplasmic antibodies.
abstract: A 43-year-old woman, with a 3-month history of fatigue, anaemia and swollen lymph nodes, underwent biopsy of a lymph node, which revealed reactive lymphadenopathy. Due to an increased serum creatinine concentration and severe proteinuria, a kidney biopsy was performed, which revealed diffuse, segmental, proliferative, immune-complex glomerulonephritis with crescents. Electron microscopy showed tubulo-reticular structures within one endothelial cell. These were a typical clinical presentation and compatible histopathological findings for systemic lupus erythematosus; however, the anti-myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) level was extraordinarily high. In spite of treatment with intravenous cyclophosphamide and methylprednisolone pulse therapy, the patient's kidney function declined. Starting plasma exchange improved her renal function and removed MPO-ANCAs, which were suspected to play the major role in the pathogenesis of glomerulonephritis. These findings indicate that in addition to lupus nephritis, MPO-ANCAs may be involved in the pathogenesis of glomerulonephritis and that the coincidence of systemic lupus erythematosus and ANCA may be responsible for the severe clinical course in our patient.
authors.affiliation: Division of Nephrology, Kantonsspital Baselland, Liestal, Switzerland ; Department of Nephrology, University Hospital Basel, Basel, Switzerland.
authors.forename: Katrin F
authors.initials: KF
authors.lastname: Koenig
authors.affiliation: Division of Nephrology, Kantonsspital Baselland, Liestal, Switzerland.
authors.forename: Stefan A
authors.initials: SA
authors.lastname: Kalbermatter
authors.affiliation: Institute for Pathology, University Hospital Basel, Basel, Switzerland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Menter
authors.affiliation: Medical Outpatient Department, University Hospital Basel, Basel, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Mayr
authors.affiliation: Division of Nephrology, Kantonsspital Baselland, Liestal, Switzerland.
authors.forename: Denes
authors.initials: D
authors.lastname: Kiss
keywords: Lupus nephritis; MPO-ANCA; Plasma exchange