BACKGROUND Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease for which there are no available laboratory parameters to clearly define the disorder. Therapy is often difficult and various treatment options have been proposed. In this paper, we have evaluated the most effective therapies for InH-AAE on the basis of current literature and report the therapeutic effect of...

Hereditary angioedema (HAE) is a rare genetic disease characterized by episodic subcutaneous or submucosal swelling. The primary cause for the most common form of HAE is a deficiency in functional C1 esterase inhibitor (C1-INH). The swelling caused by HAE can be painful, disfiguring, and life-threatening. It reduces daily function and compromises the quality of life of affected individuals and ...

INTRODUCTION Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor. CASE REPORT A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Additional analysis showed that in the IgM ...

BACKGROUND With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infe...

C1 esterase inhibitor (C1-INH) can inhibit multiple pathways (complement, contact-kinin, coagulation, and fibrinolysis) that are all implicated in the pathophysiology of asthma. We explored the effect of human plasma-derived C1-INH on allergic lung inflammation in a house dust mite (HDM) induced asthma mouse model by daily administration of C1-INH (15 U) during the challenge phase. NaCl and HDM...

Prophylactic treatment in the management of HAE is common. Berotralstat an oral once-daily selective plasma kallikrein inhibitor that was shown to reduce attack rates a Phase 3 study (NCT03485911). This post hoc analysis evaluated efficacy berotralstat patients previously treated with prophylactic medications. A total 121 were randomized 110 mg:150 mg:placebo daily for 24 weeks. Investigator-co...

Results Forty two patients, male (45%), age mean 27.1±16.9 years. Symptoms started at median age 14 years (range 1 to 58ys.). Thirty seven (86%) had familial history of HAE. They had a median of 2 episodes/mo (range 0.2 to 30) lasting 3±1.7 days/episode. Edema of limbs, face, genital and laryngeal, abdominal pain, diarrhea and vomiting were seen in 73.8%, 52.4%, 23.8%, 17%, 81%, 24% and 38.1%, ...