A case report recently published in the American Journal of Perinatology Reports aims to review the outcomes of pregnancy in women with idiopathic pulmonary fibrosis (IPF) to help guide patient counseling. The report describes a woman aged 35 years (gravida 1, para 0) diagnosed with familial IPF with no oxygen requirement.
Significant morbidity and mortality have been reported in women with IPF who become pregnant. Data on the impacts of pregnancy on IPF and maternal outcomes are limited, with the disorder typically presenting in the fourth to sixth decade of life.
IPF is a progressive restrictive lung disease resulting from an abnormal proliferation of mesenchymal cells, varying levels of fibrosis, deposition of collagen and extracellular matrix, and distortion of pulmonary architecture and subpleural cystic airspaces. According to the authors, “familial IPF is a rare form of chronic interstitial lung disease that is believed to be autosomal dominant with variable penetrance.”
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Several changes in respiratory function take place in pregnant women. These include decreased functional capacity by approximately 20% toward the latter half of pregnancy because of a reduction in both residual volume and expiratory reserve volume. Additionally, minute ventilation at rest increases by almost 50% at term, due primarily to increased tidal volume.
The patient in this study was referred to the Division of Maternal-Fetal Medicine at Eastern Virginia Medical School in Norfolk for the management of pregnancy with IPF. Because her mother had a lung transplantation at 60 years of age for IPF, the patient’s condition was assumed to be familial but without a specific genetic etiology having been identified.
Learn more about IPF etiology
Prior to pregnancy, the woman’s physical examination showed no evidence of pulmonary hypertension. She had no oxygen requirement and exercised 5 times per week. The patient did report some shortness of breath with exertion, but this resolved immediately with rest. She had undergone a preconception consultation and was counseled on the uncertain effect of pregnancy on her pulmonary function.
At 5 weeks’ gestation, the patient had established prenatal care. At this time, she noticed occasional episodic desaturation but did not require oxygen supplementation. By 22 weeks’ gestation, she stopped working, required intermittent oxygen supplementation because of desaturation with activity, and was placed on modified bed rest at home.
At 26 weeks’ gestation, the patient required 4 to 5 liters of oxygen with activity. She was ultimately hospitalized at 27 weeks’ gestation with acute hypoxic respiratory failure, with increasing shortness of breath and episodes of oxygen desaturation as low as 80% reported with minimal activity.
At hospital admission, she had a negative COVID-19 polymerase chain reaction test and viral respiratory panel. Betamethasone was administered for fetal lung maturity, and a 2-week course of intravenous methylprednisolone (40 mg daily) was initiated. Following a multidisciplinary team review, the decision was made to proceed with a cesarean section at 28 weeks’ gestation because of her worsening respiratory status.
The patient underwent an uncomplicated primary low transverse cesarean section, along with bilateral tubal ligation, under epidural anesthesia. She delivered a live male newborn weighing 1304 g (less than 3 lbs). The child was sent to the neonatal intensive care unit, where he spent the next 63 days. After 63 days, he was sent home with a good prognosis. The mother had been sent home on postoperative day 6 with an oral prednisone taper.
At 5 months’ postpartum, she continues to be oxygen dependent, with an oxygen requirement of 3 to 5 L/min despite receiving multiple courses of high-dose prednisone. If the patient does not demonstrate significant improvement in the next few months, her pulmonologist and the lung transplant team intend to proceed with lung transplantation.
“It is important for providers to be aware that data regarding pregnancy outcomes in women with chronic interstitial lung disease due to connective tissue disorders is not applicable to patients with IPF and that based on limited available data, significant maternal morbidity and mortality has been reported for women with IPF who become pregnant,” the authors wrote.
Reference
Horgan R, Kassem Z, Too G, Abuhamad A, Warsof S. Pregnancy in a patient with idiopathic pulmonary fibrosis: a case report. AJP Rep. 2023;13(1):e1-e4. doi:10.1055/s-0043-1760758
