Primary biliary cirrhosis presenting with ascites and a hepatic hydrothorax: a case report
Abstract
Introduction: We report an unusual presentation of primary biliary cirrhosis.
Case presentation: We present the case of a 66-year-old white British woman who presented to the Accident and Emergency department with ascites and unilateral pleural effusion (hepatic hydrothorax). Following a liver biopsy, the diagnosis of primary biliary cirrhosis was made.
Conclusion: It is important to consider the diagnosis of hepatic cirrhosis when presented with a unilateral pleural effusion in both the presence and absence of ascites.
Introduction
Primary biliary cirrhosis (PBC) occurs when interlobular bile ducts are damaged by chronic granulomatous inflammation. This causes progressive cholestasis, cirrhosis and portal hypertension. Often asymptomatic, diagnosis usually comes about following the discovery of a raised alkaline phosphatase on routine liver function tests (LFTs). Symptoms include lethargy and pruritis. Clinical signs include jaundice, skin pigmentation, xanthelasma, xanthomata, hepatomegaly and splenomegaly. Complications of the disease include osteoporosis, malabsorption of fat soluble vitamins, portal hypertension, ascites, variceal haemorrhage and hepatic encephalopathy. Hepatic hydrothorax is a pleural effusion occurring in patients with cirrhosis and without cardiac or pulmonary disease. It is a relatively uncommon complication and occurs when ascitic fluid moves from the peritoneum into the pleural space. The fluid movement occurs through defects in the diaphragm.
Case presentation
A 66-year-old woman presented to the Accident and Emergency department following three episodes of melaena and one episode of haematemesis. On further questioning, she also reported a 4-month history of worsening shortness of breath, initially when using stairs and more recently, after walking only a few yards. In addition to this, she admitted to abdominal distension over a number of months and weight loss of approximately 2 stone (approximately 13 kg) over the period of a year. She had no significant previous medical history and was not taking any regular medications. She was an ex-smoker, only drank occasional alcohol and was fully independent but recently had to rely on her husband for help with domestic chores due to her shortness of breath. On examination, she was noted to be tachypnoeic, tachycardic but with normal oxygen saturation and blood pressure. A cardiovascular examination was normal. Breath sounds were reduced in the entire right lung field, while the left lung was normal. Her abdomen was distended with tenderness on deep palpation in the left iliac fossa. There was no organomegaly. Shifting dullness was present and bowel sounds were normal. A digital rectal examination demonstrated a prolapsed rectum with fresh blood and dark stool on the finger tip. She was alert and fully orientated.
