Immune thrombocytopenic pupura

Given the limited real-world data of caplacizumab, our multicenter real-world study was designed to assess the safety and efficacy of caplacizumab in immune thrombotic thrombocytopenic pupura (iTTP), compared to historic controls. We have studied 70 patients: 23 in the caplacizumab and 47 in the historic control group. Plasma exchange was applied in all episodes except for two patients that denied plasma exchange. Rituximab as first-line treatment was more common in the caplacizumab group compared to historic control...

Background: Previous study revealed proton pump inhibitors (PPIs) have an effect on gut microbiota. Alteration of the microbiome causes changes of the host immune system and then induces the development of autoimmune diseases (ADs). This study aimed to explore the possible association between PPIs use and ADs. Methods: This study was conducted using data from the Taiwan National Health Insurance Research Database in the period between 2002 and 2015. We performed multivariate and stratified analysis through the Kaplan-Meier method and Cox proportional hazard models to estimate the association between proton pump inhibitor use and the risk of autoimmune diseases...

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by mechanical hemolytic anemia, profound thrombocytopenia, and neurological manifestations. Acquired auto-immune TTP, the most prevalent cause of TTP, is induced by the presence of inhibitory anti-ADAMTS13 auto-antibodies. Modern treatment of acquired TTP relies on plasma exchange, rituximab, and steroids. Caplacizumab (Cablivi®), a humanized single-variable domain immunoglobulin that targets the A1 domain of the ultra-large von Willebrand factor, inhibits the interaction between ultra-large vWFand platelets...

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Intravenous immunoglobulins (IVIgs) exert a variety of immunomodulating activities and are therefore increasingly being used for the treatment of immune-mediated as well as autoimmune diseases. Although the exact mode of action is still poorly understood, the efficacy of IVIg in several diseases, such as thrombocytopenic pupura and Kawasaki disease, has been proven in clinical trials. According to some uncontrolled clinical studies and many case reports there is evidence for a high efficacy of IVIg in the treatment of skin diseases such as autoimmune blistering diseases, lupus erythematosus, dermatomyositis, scleroderma, toxic epidermal necrolysis, atopic dermatitis, chronic autoimmune urticaria and others...