Diagnosis & Disease Information

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Long-Term Prophylaxis With C1-INH Could Help Reduce the Burden of HAE

Reducing the burden of hereditary angioedema (HAE) could be possible with adequate dosing and frequency of administration of C1 inhibitor (C1-INH), even for patients with highly symptomatic HAE activity, according to data from randomized controlled trials evaluating the administration of C1-INH replacement therapy as long-term prophylaxis in patients with HAE. Up to 83% of patients…

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Clinicians Face Particular Challenges When Treating HAE in Rural Areas

The diagnosis and management of patients with hereditary angioedema (HAE) who reside in rural communities present particular challenges, according to findings from an online survey conducted in the United States and published in the Annals of Allergy, Asthma & Immunology. The researchers sought to explore the experiences of allergy and immunology health care professionals in…

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Fatigue in AAV May Meet Criteria for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

A sizeable portion of individuals with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) experience fatigue severe enough to meet the diagnostic criteria for myalgic encephalomyelitis/chronic fatigue syndrome, according to a study published in The Lancet Regional Health — Americas. The authors of this study sought to assess fatigue in patients with AAV to see if it…


A Case of AAV With Gastrointestinal System Involvement

A patient with granulomatosis with polyangiitis, a form of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), received long-term multidisciplinary treatment at an intensive care unit (ICU) due to massive hemorrhage and multisystem involvement, according to an article published in the Turkish Journal of Trauma and Emergency Surgery. The study authors reported on a 37-year-old male patient…

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Higher Mortality and Infection Rates Seen in Patients With AAV and ESKD

In patients with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and end-stage kidney disease (ESKD) who are receiving renal replacement therapy, higher rates of mortality and infection have been reported, along with a lower risk for relapse, according to findings from a meta-analysis published in Seminars in Arthritis and Rheumatism. Approximately 20% of patients with AAV…

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CanVasc Publishes Updated Recommendations for Avacopan Use in AAV

The Canadian Vasculitis Research Network (CanVasc) has updated its consensus recommendations for the use of avacopan in patients with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). The guidelines, published in Rheumatology, are based on data confirming that avacopan is an effective steroid-sparing therapy for microscopic polyangiitis and granulomatosis with polyangiitis. “Prospective studies published between 2017 and…

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Dr. Federico Stella on Using Nonrestrictive Diets After Stem Cell Transplantation

Larry Luxner, senior correspondent for Rare Disease Advisor, interviews Federico Stella, MD, on the controversial practice of severely restricting the diets of patients who have just undergone stem cell transplants. Read the full story here: Italian Hematologist Urges End to ‘Useless’ Restrictive Diets After Stem Cell Transplants

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New Study Set to Assess Povetacicept in Adults With CAD and Other Cytopenias

Alpine Immune Sciences is launching a new study to evaluate povetacicept (ALPN-303) in adults with autoimmune cytopenias including immune thrombocytopenia, warm autoimmune hemolytic anemia, and cold agglutinin disease (CAD). The study, listed on ClinicalTrials.gov, aims to determine whether povetacicept is safe and potentially effective for treating these conditions. CAD is a rare autoimmune disease characterized…

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