Hepatopulmonary syndrome caused by sarcoidosis of the liver treated with transjugular intrahepatic portosystemic shunt
- 1Universitätsklinikum Hamburg-Eppendorf, I. Medizinische Klinik und Poliklinik, Hamburg, Germany
- 2Universitätsklinikum Hamburg-Eppendorf, II. Medizinische Klinik und Poliklinik, Hamburg, Germany
- Correspondence to Dr Markus W Nistal, Universitätsklinikum Hamburg-Eppendorf, I. Medizinische Klinik und Poliklinik, Martinistr. 52, 20246 Hamburg, Germany;
- Received 4 October 2011
- Accepted 1 February 2012
- Published Online First 1 March 2012
- hepatopulmonary syndrome
- transjugular intrahepatic portosystemic shunt
- respiratory infection
Resident: I would like to present the case of one of my new patients. She is a 63-year-old Caucasian woman with long-term sarcoidosis of the lungs and multiorgan involvement including liver and spleen. Despite pneumological surveillance and prednisolone treatment, her respiratory situation worsened, and was started on home oxygen treatment 5 years ago when fibrosis of the lungs was diagnosed. She also has severe osteoporosis due to long-term steroid use and immobility. She is now presenting with worsening dyspnoea of a duration of 4 months despite using 8 litres of oxygen.
I admitted a prematurely aged woman in a poor general condition. She presented with dyspnoea, right upper quadrant abdominal pain and back pain. A physical examination revealed stigmata of hypoxaemia, light crackles on both lungs, clinical signs for steroid use and an enlarged liver painful on palpation. She is a non-smoker and she has no history of cardiac diseases or cardiac involvement by sarcoidosis. An echocardiogram was normal and right heart catheterisation 2 months ago showed no pulmonary hypertension. There was no fever or relevant inflammation parameters. Laboratory tests revealed a mild hyperbilirubinaemia of 1.8 mg/dl, slightly reduced albumin of 32 g/litre, normal transaminases and an elevated γ-glutamyl transpeptidase (GGT) (981 U/litre) and alkaline phosphatase (AP) (559 U/litre). Her International Normalised Ratio (INR) was within the normal range. An ultrasound scan showed an enlarged liver and spleen and some ascites.
Consultant: Concerning her respiratory situation, we have to think of a worsening of lung fibrosis due to sarcoidosis, since there is no trace of cardiac or infectious conditions so …