Pulmonary hypertension associated withprimary biliary cirrhosis intheabsence ofportal hypertension: acasereport

Pulmonary hypertension iswelldescribed in association withportal hypertension ofany causeincluding endstageprimary biliary cirrhosis (PBC). Theessential feature ofthis association isthepresence ofportosystemic shunting, including surgically created shunts. A patient withprimary pulmonary hypertensionandPBCwithout portal hypertension is reported. Thissuggests thatprimary pulmonaryhypertension maybeassociated with PBCintheabsence ofportal hypertension. Decisions regarding appropriate organtransplantation maydepend onwhether pulmonary hypertension isprimary orsecondary toportal hypertension. (Gut1994; 35:280-282) Primary biliary cirrhosis (PBC)isa chronic disorder whereinflammation andfibrosis of intrahepatic bile ducts leads tocirrhosis andliver failure. PBChasbeenassociated withnumerous diseases ofanautoimmune nature, mostnotably, thyroiditis andvarious rheumatological manifestations. 'Pulmonary involvement inPBC includes interstitial lungdisease andairways obstruction.23 Pulmonary hypertension (PHT) hasbeendescribed only withendstage PBC4as wellasportal hypertension ofanycause. We present apatient withPHT andPBCinthe absence ofdemonstrable portal hypertension, suggesting thatprimary pulmonary hypertension maybeindependently associated withPBC.