[Systemic disease with hepatic and pulmonary involvement].

We discuss the history of a 39 year old woman with multiple patchy consolidations on high - resolution computed tomography in combination with a delayed diagnosis of primary biliary cirrhosis. Further we review the differential diagnostic considerations and step by step diagnostic and therapeutic options. Based on the presentation with symptoms of a mitigated pneumonia at the beginning of the disease, the differential diagnosis of cryptogenic organising pneumonia (CO) is discussed. The diagnosis of COP is often delayed. The theoretic background of primary biliary cirrhosis and the association with pulmonary disease are broadly discussed.