Facebook
Twitter
LinkedIn
Email
 |
Checking for direct PDF access through Ovid | |
Excerpt
Goodpasture syndrome, an immunologic disorder characterized by glomerulonephritis and pulmonary hemorrhage, is rare in pregnancy but can cause significant maternal and fetal/neonatal morbidity. In this case, a 34-yr-old gravida 2, para 0, presented at 18 wk gestation with malaise, nausea, vomiting, diarrhea, pruritus, decreased urine output, foamy urine, occasional coughing, mild hemoptysis, and a history of respiratory illnesses, including chronic eosinophilic pneumonia with nonnecrotizing vasculitis and alveolar hemorrhage. On presentation this time, findings on physical examination were normal, but the hemoglobin level was 7.8 g/dL, serum creatinine 1306 μM, and potassium 7.6 mM, with peaked T waves. She was dialyzed urgently, transfused, and transferred to a tertiary perinatal center.
At this point, she had proteinuria, trace glucosuria, and hematuria; and a renal biopsy indicated rapidly progressive glomerulonephritis. Treatment included daily hemodialysis, plasmapheresis, methylprednisolone, and prednisone. At 23 wk, azathioprine was added because of streaky hemoptysis and prednisone was tapered. Ten days later, she had normal lung function, but her blood pressure continued to be high, despite alpha-methyldopa therapy. Complete blood counts, renal function, and electrolytes were monitored regularly and anemia was managed with erythropoietin. At 25 wk, the fetal status became nonreassuring, and after expectant management, cesarean section (CS) was performed at 26 4/7 wk. A male neonate weighing 700 g was delivered in stable condition; at 36 h, however, cranial ultrasound showed massive intraventricular and intraparencymal hemorrhage, with continued ventricular enlargement and development of a communicating hydrocephalus. Placental pathologic exam showed low placental weight; extensive thrombotic vasculopathy; basal plate hematoma; and subchorionic, intervillous, and interlobular septal hematomas. Neurodevelopmental follow-up at 12 mo revealed a global delay, generalized hypotonia, and exaggerated refiexes; since then, the infant has shown failure to thrive. After delivery, the mother was treated with erythropoietin, calcium, vitamin D, azathioprine, and a prednisone taper; and hemodialysis was discontinued. Renal function, creatinine, protein, and total urine output improved to normal levels, with hypertension controlled with metoprolol, amlodipine, and ramipril.
Based on these findings and those of four previous cases, the authors concluded that preconception disease presentation and initiation of treatment can lead to a favorable neonatal outcome, whereas presentation during pregnancy increases risk of fetal/neonatal morbidity. Maternal outcome is less predictable and may depend on therapy and patient compliance. Maternal compromise can arise from pulmonary or renal complications, and invasive measures (renal biopsy, hemodialysis) may be required and early termination warranted.
