Pregnancy in a patient with primary sclerosing cholangitis

Introduction:

Primary sclerosing cholangitis (PSC) is a progressive disease of unknown etiology. It is characterised by ongoing inflammation, fibrosis, and destruction of bile ducts finally resulting in liver failure. A rare case of PSC in pregnancy is presented.

Case presentation:

A 19 year-old woman gravida 1, para 0, was diagnosed with PSC five years before her pregnancy. Besides PSC, her past medical history revealed ulcerative colitis. During her pregnancy in the first trimester cholestatic liver parameters were considerably elevated, which normalised in the second trimester. The patient was admitted to the hospital at 27 weeks of gestational age for obstetrical care. During hospitalisation, we observed a flattening of the fetal growth curve and arterial redistribution. At 33 weeks the patient developed a severe acute cholangitis and the pregnancy was terminated by cesarean section. Postpartum liver functions worsened and the patient underwent an endoscopic therapy including balloon dilatation and stent placement for subhiliar bile duct stenosis.

Conclusion:

PSC coincidental with pregnancy is rare. Nevertheless, the outcome can be favorable. Therapy is aimed at treating symptoms und managing complications. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease.