Der Internist
-
The acute porphyrias are a group of four metabolic defects in which the heme synthesis in the liver is disrupted. They are characterized by massively painful acute attacks, which can be life-threatening if not diagnosed. To raise the awareness for these rare disorders, a heme molecule in cartoon style is introduced, which accurately explains the basic biochemical processes in the body and mediates important information on the acute hepatic porphyrias in a simplified and attractive way. The article is complemented by a case report.
-
In addition to conventional anti-inflammatory treatment for chronic inflammatory bowel disease (IBD), there has been an evolution of new treatment options over the past 20 years. Already approved biologics provide multiple treatment alternatives but also make the treatment algorithms more complex. This development results in a substantial improvement in patient care. The ambitious treatment targets are associated with a higher quality of life and the reduction of long-term disability and morbidity. ⋯ The diversity of IBD treatment offers increasing treatment options and thus improved patient care; however, as the number of new substances increases treatment becomes more complex. This article summarizes the current and future treatment options for IBD and their integration into current treatment algorithms.
-
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of rare chronic autoimmune diseases characterized by recurrent systemic inflammation provoking multiple morbidities. AAV patients suffer from various organ manifestations and treatment-related severe adverse effects. This retrospective study investigated the concrete burden of AAV disease on patients in Germany. ⋯ Based on this analysis of German health care data, disease-specific assumptions regarding the burden on AAV patients were confirmed and concretized for the German context. AAV patients suffer from a high burden of morbidity, including multiple disease manifestations, relapses, and severe complications due to AAV treatment.
-
Patients with atrial fibrillation are at a significantly increased risk of thromboembolic events, especially ischemic strokes. Oral anticoagulation reduces this risk, but cannot be used in some patients for various reasons and is associated with a relevantly increased risk of bleeding. As an alternative for prophylaxis of thromboembolic events in patients with atrial fibrillation, there are different options of left atrial appendage closure. ⋯ In carefully selected patients suffering from atrial fibrillation with relative or absolute contraindications for oral anticoagulation, interventional closure of the atrial appendage is a safe alternative for prophylaxis against thromboembolic events. The currently available scientific evidence from randomized controlled trials is sparse. Nevertheless, extensive amounts of registry study data suggest a benefit, while the results of several large randomized controlled trials are expected in the coming years.
-
Despite therapy with glucocorticoids (GC) and conventional immunosuppressants, patients with connective tissue diseases and vasculitides often develop functionally relevant and prognostically unfavourable internal organ damage. Based on new pathogenetic insights, biologics and small molecules have recently been studied as targeted therapies for collagen vascular diseases and vasculitides. The B lymphocyte stimulator antagonist belimumab has been used for the treatment of systemic lupus erythematosus (SLE) for several years and has recently also been approved as an add-on therapy for lupus nephritis. ⋯ In patients with EGPA, the IL‑5 antibody mepolizumab leads to improved disease control and reduces GC requirements. A phase III trial of the small molecule antagonist avacopan targeting the complement C5a receptor as a replacement for high-dose GC in induction therapy of GPA and MPA met its primary endpoints. Various other biologics and small molecule antagonists are currently in clinical development for several type of vasculitis and collagen vascular diseases, some of them at advanced stages.