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For nearly 30 years ursodeoxycholic acid (UDCA) represented the only pharmacological treatment option available for primary biliary cholangitis (PBC). This changed at the end of 2016 when obeticholic acid was licensed in Europe for PBC patients not responding to UDCA. Novel treatment concepts involving the modulation of nuclear receptor signaling in cholestatic and other liver diseases have led to a host of new potential options, studies and drug candidates for the treatment of PBC. The analysis of large multinational cohorts has additionally confirmed the effectiveness of UDCA in slowing PBC progression, and has led to the development of new definitions for the risk assessment of PBC patients under therapy, which will be an asset for clinical decision making. One issue that remains unresolved is the therapeutic management of extrahepatic symptoms associated with PBC, namely fatigue and pruritus, which are the main factors influencing the quality of life of affected individuals. Their pathophysiological basis is poorly understood and treatment remains unsatisfactory.

A 59-year-old woman suffered from fever and upper abdominal pain. The computed tomography (CT) scan revealed a liver lesion. Conventional imaging techniques (CT, magnetic resonance imaging, contrast-enhanced ultrasonography) did not allow for a consistent diagnosis. Fine needle biopsy of the liver lesion was performed. Histologically, fibrotic inflammation was found and an inflammatory pseudotumor (IPT) diagnosed. Despite treatment with steroids and antibiotics, the size of the IPT increased; thus, surgical resection was necessary. In case of fever of unknown origin, IPT should be considered as a potential diagnosis.

Recent non-cirrhotic and non-malignant splanchnic vein thrombosis is now defined as extrahepatic portal vein thrombosis with or without involvement of the mesenteric vein according to the Baveno VI consensus from 2015. An early diagnosis is often challenging due to unspecific symptoms with abdominal pain or diarrhea but extremely important because of the potential acute and chronic complications, such as mesenteric ischemia and portal hypertension; therefore, rapid treatment is crucial. We present two cases of severe splanchnic vein thrombosis, which were treated with catheter-directed local thrombolysis and thrombus aspiration. These minimally invasive catheter-directed techniques have recently been successfully used in selected patients. A review of the literature is provided in this article. In summary, the management of splanchnic vein thrombosis must be individually planned for each patient and should be performed at experienced centers, which can provide all therapeutic options. In selected cases with the correct indications transjugular transhepatic catheter-directed local thrombolysis is a safe option with a good outcome.

Donor organs for transplantations are a scarce commodity; therefore, allocation systems are needed that guarantee an ethically acceptable distribution to patients on the waiting list (equal treatment and fairness) but also take the probability of survival of the transplant in each recipient into consideration. In this article the allocation systems for lung, liver, kidney and pancreas transplants are presented.For lung transplantations an allocation system based on the lung allocation score (LAS) is currently used. The LAS predicts the probability of survival on the waiting list and the survival rate following transplantation. Organs with a limited range of utilization are distributed in a so-called mini-match procedure.For post-mortem kidney and pancreas transplantations a relatively complex but transparent allocation system has been created in which patients are subdivided into groups, each of which has its own allocation rules. The allocation is principally carried out according to criteria of fairness of distribution and according to the prospects of success. The probability of a mismatch also plays a role. The urgency is important for children and for patients who do not have the possibility of dialysis. Combined pancreas and kidney transplantations have priority over kidney transplantations alone.The criterion for the urgency of liver transplantation in Germany is currently the model for end-stage liver disease (MELD), which aims to reduce the waiting list mortality and to prioritize transplantations for those most in need. Because the system insufficiently describes the priority of transplantation for patients with tumors or genetic liver diseases, there is an additional set of rules for so-called standard exceptions.

Since the 1950s, autoimmune hepatitis has been recognized as a chronic liver disease that responds to treatment with steroids. Nevertheless, even today 25% of all diagnosed patients still present with liver cirrhosis, which emphasizes the importance of a timely and efficient diagnosis. Steroid-containing immunosuppression is characterized by a high rate of unwanted side effects. For non-cirrhotic patients, the synthetic steroid budesonide, which is characterized by high first-pass metabolism, represents a study-evaluated alternative. In the new guidelines for the management of primary sclerosing cholangitis, the therapeutic role of ursodeoxycholic acid has been modified, with no other effective alternative drugs being available. Management of sclerosing cholangitis is also made more difficult by the high risk of cholangiocarcinoma and colon carcinoma in addition to new differential diagnoses such as secondary sclerosing cholangitis and IgG4-associated cholangitis. IgG4-associated cholangitis can mimic primary sclerosing cholangitis but--in contrast-- can respond to the administration of steroid-containing immunosuppression.

Hepatopulmonary syndrome, portopulmonary hypertension and hepatic hydrothorax are typical pulmonary complications in patients with liver cirrhosis. Whereas hepatopulmonary syndrome and portopulmonary hypertension represent pulmonary vascular diseases, the development of hepatic hydrothorax is associated with the presence of ascites and phrenic lesions. For severe hepatopulmonary syndrome and refractory hepatic hydrothorax, liver transplantation is the treatment of choice. In severe portopulmonary hypertension specific medical treatment is indicated. In selected patients, beside intravenous prostanoids, oral endothelin receptor antagonists and phosphodiesterase type-5 inhibitors are possible treatment options.

In Germany, around 800.000 to 1 million individuals are chronically infected with either the hepatitis B or the hepatitis C virus. Viral hepatitis is therefore highly relevant for the everyday management in clinical practice. For the treatment of hepatitis B, potent antiviral drugs have become available that, in the majority of patients, induce viral suppression. This requires a strategic therapeutic planning in view of a likelihood of long term administration and the prevention of viral resistance. Recent advances in the treatment of hepatitis C are based on a therapeutic individualization based upon viral kinetics and genotypes that also requires strategic planning. In contrast to hepatitis B, viral elimination can be reached in 50-90% of hepatitis C patients. Hepatitis D is associated with a migration background, is most likely under diagnosed and does not yet have a satisfactory curative treatment option. An effective vaccine exists for hepatitis A that offers protection lasting decades. Hepatitis A does not lead to chronic infection. This was also assumed for hepatitis E. However, reports indicate chronic courses in selected patient groups. A vaccine for hepatitis E is currently not yet available.

After around 64,000 transplantations in Europe since 1988 liver transplantation has emerged as a standard treatment option for otherwise incurable chronic liver diseases. Cirrhosis of different etiologies represents the most frequent transplant indication. Overall survival in this group amounts to 72% after 5 years, and 62% after 10 years. In Germany, the main indications include alcoholic liver cirrhosis, tumors with increasing numbers in recent years, as well as viral diseases leading to cirrhosis. Since December 2006 the priority for liver transplantation is determined by the model for end stage liver disease (MELD) and not by the length of waiting time. MELD is a statistical model based on serum creatinine, serum bilirubin and coagulation, which describes the probability of 3-month mortality of a potential transplant candidate. Not all liver diseases are adequately represented by MELD necessitating the additional use of a defined number of standard exceptions that have been last updated in 2008. As a consequence of these developments indications, selection of recipients and the management of the waiting list have seen profound change.