A Case Report of Hepatic Cirrhosis with Portal Hypertension Secondary to Alpha-1 Antitrypsin Deficiency And Autoimmune Hepatitis In Pregnancy

The prevalence of cirrhosis among women of reproductive age is rising, leading to more pregnancies in this population despite historical challenges related to fertility and hormonal dysfunction. Autoimmune hepatitis (AIH), which is more common in women, can lead to cirrhosis and poses risks during pregnancy, including severe flares of the condition. Treatment typically involves medications like Prednisolone and Azathioprine, but women with AIH face increased risks of complications such as preeclampsia, hepatic decompensation, maternal death, early fetal loss, prematurity, low birth weight, and a high rate of Caesarean sections.

The present case reported a 29-year-old woman with cirrhosis and portal hypertension who became pregnant unexpectedly. She had a history of was known to have cirrhosis secondary to homozygous alpha-1 antitrypsin deficiency and associated autoimmune hepatitis. She was treated in the past with several therapeutic agents including Prednisolone, Azathioprine, Spironolactone, Frusemide, and Propranolol, however all were stopped by herself due to side effects. She also experienced 4-5 episodes of variceal bleeding prior to her pregnancy. An endoscopy revealed grade 3 varices at the time of her most recent presentation of variceal hemorrhage a year earlier. She has stopped smoking and drinking alcohol upon confirming that she was pregnant.

On her first appointment, she was normotensive (BP 120/70) and weighed 84.6 kg (BMI 27.8). She had marked peripheral edema and splenomegaly (span ~20 cm). The liver was not palpable, there was no ascites and no spider nevi were present.

At 18 weeks when she presented to the ED with complaints of abdominal pain, nausea and hematemesis. She underwent banding of her eseophageal varices and non-bleeding gastric varices were also identified during this period. She was prescribed Propranolol, intravenous Pantoprazole and a unit of packed red blood cells as her Hemoglobin was 72 g/L. A multidisciplinary team managed her care throughout gestation, monitoring her condition closely.

Her MRI revealed a significant cirrhotic liver with evidence of portal hypertension and extensive perigastric and paraesophageal varices extending into the mediastinum. She had massive splenomegaly with the spleen spanning at least 250mm in craniocaudal length. Complications included the need for endoscopic procedures to address varices and had two elective endoscopies at 27 and 30 weeks. Non-bleeding esophageal varices were identified and banded on both occasions.

She ultimately delivered via elective Caesarean section at 32+6  weeks due to concerns about bleeding risks and gave birth to a liveborn female weighing 2269 g (82nd centile) with Apgars of six and eight, at one and five minutes respectively.

Post-delivery, the patient experienced fluid overload but was stable and received follow-up care. However, the patient's liver function deteriorated two weeks postpartum, leading her hepatologists to initiate treatment with Prednisone. Unfortunately, after only two doses, she experienced worsening symptoms and had to stop the medication. At six weeks post-delivery, a follow-up endoscopy showed non-bleeding grade 2 esophageal varices, which were banded, and bulging at the gastric fundus, likely indicating paragastric varices, 

Thus, the report highlighted the importance of a multidisciplinary approach and diligent antenatal care in managing pregnancies complicated by liver disease, as well as the need for further research on pregnancy outcomes in women with cirrhosis and portal hypertension. it emphasizes the rising number of pregnancies in this group and the associated riks, which must be carefully managed to optimize both maternal and fetal health.

Source: Galibert S, O’Rourke N, Wolski P, Schmidt B. Hepatic cirrhosis with portal hypertension secondary to alpha-1 antitrypsin deficiency and autoimmune hepatitis in pregnancy: A case report. J Case Rep Images Obstet Gynecol 2022;8(2):38–44.