Abstract: TH-PO632

Microscopic Polyangiitis with Pulmonary Renal Syndrome in a Pregnant Woman: Management Challenges

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Kandavar, Ramprasad, Harbor-UCLA Medical Center, Torrance, California, United States
  • Kommana, Sandhya L, Harbor ULCA MEDICAL CENTER, Harbor City, California, United States
  • Shah, Anuja P., Los Angeles Biomedical Research Institute at Harbor-UCLA, Torrance, California, United States
Background

Introduction
Microscopic Polyangiitis occurring either de novo or relapse during pregnancy has significant maternal and fetal morbidity and mortality. Here we report a case of pregnant woman developing de novo MPA manifesting as pulmonary renal syndrome posing considerable management challenges

Methods

Case Description
A 21year-old pregnant woman (G1P0) presented to the Emergency department at 21 weeks gestation with uncontrolled blood pressure, pulmonary edema and acute renal failure. Laboratory data significant for Hemoglobin of 6.4, BUN 49 and Serum Creatinine 7.15 mg/dl. UA showed 3+protein, 3+ blood with numerous Acanthocytes. Further studies revealed P- ANCA 1:160 and Anti MPO: 24. Renal US was revealed normal sized kidneys. Early clinical course also complicated by hemoptysis suggestive of pulmonary hemorrhage. A diagnosis of RPGN secondary to Microscopic Polyangiitis was made. She was started on pulse steroids followed by oral Prednisone, IV cyclophosphamide, Hemodialysis 6 times/week and Plasmapheresis.Over the course of several days patient made significant clinical stabilization,hemoptysis resolved and ANCA and anti-MPO titers became negative. Patient was managed in close consultation with Obstetrics Service and underwent Cesarean section at 27.2 weeks. Patient received monthly infusion of Cyclophosphamide with no signs of renal recovery and currently is dependent on hemodialysis. Renal biopsy was done after delivery, showed immune complex mediated glomerulonephritis, chronic/inactive crescentic glomerulonephritis involving 85% of glomeruli with moderate to severe scarring.

Conclusion

Discussion:
The data on MPA in pregnancy and its outcomes is very limited and is thought to have a more aggressive course. A systematic review of 48 pregnancies with small vessel vasculitis showed 33 % prematurity, 8% miscarriage, pre-eclampsia 17% and maternal death 4%. Many of the immunosuppression drugs including Cyclophosphamide are contraindicated in pregnancy because of teratogenicity, ovarian failure, and fetal prematurity. However, there are anecdotal reports of Cyclophosphamide use in pregnant women with malignancies like leukemia, lymphoma and breast cancer confirming safety of the drug in pregnancy. In our case we faced similar treatment dilemmas though in the end we were able to deliver a viable fetus.

Funding

  • Other U.S. Government Support