Outcome of idiopathic pulmonary fibrosis with pregnancy

Background
Idiopathic pulmonary fibrosis (IPF) is a chronic pulmonary disease with an unknown etiology. It is uncommon in women of childbearing age. Limited data are available regarding the outcome of IPF with pregnancy.


Objectives
To evaluate both maternal and neonatal outcomes among pregnant women with IPF in comparison with pregnant asthmatic patients. Analysis of potential factors associated with unfavorable outcomes was done.


Patients and methods
This prospective case–control study recruited 20 pregnant women with IPF and 43 asthmatic pregnant controls. Demographics and disease-related parameters were recorded. Functional and radiological evaluations were performed. Watchful peripartum follow-up and precise management in a multidisciplinary team were done. Maternal and neonatal outcomes were evaluated with an analysis of prognostic factors for unfavorable outcomes.


Results
The mean age (SD) of study patients was 31.2 (6.4) years. Seven (35%) cases showed moderate functional impairment, and nine (45%) cases showed severe impairment. All cases required oxygen therapy by the third trimester. Five (25%) cases required ICU admission during the peripartum period. A total of 15 (75%) cases needed domiciliary oxygen upon discharge from the hospital (unfavorable maternal outcome) with no maternal mortality recorded. Overall, 11 (55%) cases were associated with neonatal complications (unfavorable neonatal outcome). The degree of functional impairment showed a significant association with neonatal outcome (P=0.02). Both maternal and neonatal outcomes were significantly better in the control group than in cases (P<0.001).


Conclusion
Pregnancy course in patients with IPF can be associated with adverse effects on maternal and neonatal outcomes and disease progression. However, collaborative management can minimize maternal mortality.