Prof. Drenth has a long term research interest in rare (inherited liver) diseases. During his scientific career he discovered genes for autoinflammatory diseases, inherited pancreatitis, and polycystic liver disease. He focuses on. He initiated >50 rigorous clinical trials in many rare diseases such as hereditary angiodysplasias, autoimmune disorders, polycystic liver disease, polycystic kidney disease amongst others. This opened up the road for novel treatment options for rare disease patients. Prof Drenth is the Editor-in-Chief of the UEG journal (Journal Impact Factor 6.9). His research endeavours resulted in ~600 peer reviewed articles, and he is cited widely.