Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs.
Despite clinical and experimental data suggesting a direct relationship between antineutrophil cytoplasmic antibodies (ANCAs) and disease activity in patients with microscopic polyangiitis (MPA), the causal relationship between perinuclear ANCAs specific for myeloperoxidase (MPO-ANCA) and disease manifestations has been controversial. We describe the case of a woman with a history of pulmonary-renal syndrome caused by MPA whose disease became clinically and serologically active during pregnancy. Forty-eight hours after delivery, the newborn developed pulmonary hemorrhage and abnormalities in renal function. The newborn's cord blood showed an immunoglobulin G MPO-ANCA level identical to that of the mother's serum, indicating passive transfer of the antibody to the neonate. Our findings represent the first human model supporting the interpretation that MPO-ANCAs were immunopathogenic.
Schlieben
David J
DJ
Department of Medicine, Section of Nephrology, Rush University Medical Center, Chicago, IL, USA.
Korbet
Stephen M
SM
Kimura
Robert E
RE
Schwartz
Melvin M
MM
Lewis
Edmund J
EJ
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D002585:Cesarean Section; D004417:Dyspnea / Q000209:etiology; D004630:Emergencies; D005260:Female; D005312:Fetal Blood / Q000276:immunology; D005921:Glomerulonephritis / Q000276:immunology; D006470:Hemorrhage / Q000151:congenital / Q000276:immunology; D006801:Humans; D007112:Immunity, Maternally-Acquired; D007231:Infant, Newborn; D007518:Isoantibodies / Q000276:immunology*; D007674:Kidney Diseases / Q000151:congenital* / Q000276:immunology; D008171:Lung Diseases / Q000151:congenital* / Q000276:immunology; D008431:Maternal-Fetal Exchange; D009195:Peroxidase / Q000276:immunology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000276:immunology*; D013577:Syndrome; D014657:Vasculitis / Q000151:congenital* / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D001324:Autoantigens; D007518:Isoantibodies; D009195:Peroxidase