Takayasu arteritis (TAK) is a rare, chronic large-vessel vasculitis (LVV) that predominantly affects aorta, its major branches and the pulmonary arteries. Recent advances in the genetics, clinical course, prognosis, disease assessment with biomakers/imaging/new scoring systems and new treatment options in TAK are discussed in this review. New imaging modalities such as MRAngiography (MRA) and 18-FDG-PET seem to have replaced conventional angiography for the initial diagnosis in recent years. MRA and 18-FDG-PET are also promising for the assessment of disease activity. New tools for disease assessment such as Indian Takayasu Arteritis Score (ITAS2010) and colour-Doppler ultrasonograpy score (CDUS) aim to quantify disease activity better, however different imaging modalities, used in routine follow-up, are not sufficiently incorporated in these scoring systems. Prognosis is possibly getting better with lower mortality in recent years, however there are still widely different vascular intervention rates in clinical series. Leflunomide, TNF-α antagonists and tocilizumab are new options in patients resistant to conventional therapies.