Case reports
Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs

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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.

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Case report

A 32-year-old woman with a history of MPA was admitted during the 33rd week of her second pregnancy for evaluation of shortness of breath. She had been well throughout this pregnancy; however, 1 week before admission, she developed hypertension, and on admission, she reported a 1-day history of difficulty breathing.

The patient initially presented with pulmonary hemorrhage and glomerulonephritis 8 years before admission. MPO-ANCA level was elevated (42 U/mL; normal, <7 U/mL), and a renal biopsy

Discussion

MPA is a systemic disease characterized by vasculitis involving small blood vessels, particularly the glomerular and pulmonary capillaries. Clinical manifestations include necrotizing pauci-immune glomerulonephritis and pulmonary hemorrhage.2, 3, 4, 5 Despite clinical data suggesting a direct relationship between ANCAs and disease activity in patients with MPA,9, 10, 11, 12 the causal relationship between P-ANCAs specific for myeloperoxidase (MPO-ANCA) and disease manifestation has been

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Originally published online as doi:10.1053/j.ajkd.2005.01.001 on February 22, 2005.

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