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Case based discussion
Hepatopulmonary syndrome caused by sarcoidosis of the liver treated with transjugular intrahepatic portosystemic shunt
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  1. Markus W Nistal1,
  2. Andrea Pace1,
  3. Hans Klose2,
  4. Daniel Benten1,
  5. Ansgar W Lohse1
  1. 1Universitätsklinikum Hamburg-Eppendorf, I. Medizinische Klinik und Poliklinik, Hamburg, Germany
  2. 2Universitätsklinikum Hamburg-Eppendorf, II. Medizinische Klinik und Poliklinik, Hamburg, Germany
  1. Correspondence to Dr Markus W Nistal, Universitätsklinikum Hamburg-Eppendorf, I. Medizinische Klinik und Poliklinik, Martinistr. 52, 20246 Hamburg, Germany; markus.nistal{at}web.de

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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 …

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