People with ANCA-associated vasculitis who have high levels of a protein called aminoacyl-tRNA synthetase-interacting multifunctional protein-1 (AIMP1) in their blood serum might be at higher risk of developing a severe form of the disease, a study has found. The study, “Serum Aminoacyl-tRNA Synthetase-Interacting Multifunctional Protein-1…
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Patients with ANCA-associated vasculitis (AAV) with low levels of complement proteins develop greater kidney damage, according to new research. The study, “Increased renal damage in hypocomplementemic patients with ANCA-associated vasculitis: retrospective cohort study,” appeared in the journal Clinical Rheumatology. The complement system — a…
It wasn’t until Gordana Loleska’s son David was 14 years old that doctors in their native North Macedonia diagnosed his kidney, vision, and hearing problems as Alport syndrome. Although she had known for years that something was wrong, the news that David would battle a lifelong rare disease devastated…
In the rare event that ANCA-associated vasculitis affects a large blood vessel like the aorta, treatment with Rituxan (rituximab) appears to improve symptoms and reduce inflammation, a case report shows. The study, “Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review,” was published in…
Complement System Plays Role in ANCA-associated Vasculitis, Regardless of ANCA Subtype, Study Says
The complement system, an important part of immunity, is activated in ANCA-associated vasculitis patients with myeloperoxidase (MPO) antibodies as well as those with proteinase 3 (PR3) ANCA antibodies, but its activation profile appears to differ by severity of disease and possibly by ANCA subtype, a new study shows. The…
Major infections are the main cause of poor long-term outcomes and death in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, a Spanish study found. The study, “Prevalence and Risk Factors for Major Infections In Patients with Anca-associated Vasculitis. Influence on The Disease Outcome,” was published in…
A violinist with vasculitis, two Texas politicians and a pharmaceutical company whose marijuana-derived therapy helps kids with Dravet syndrome were among winners of the 2019 Rare Impact Awards. Officials of the National Organization for Rare Disorders (NORD) presented the awards during a June 22 dinner attended by…
Neutrophils from patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are better at activating the alternative complement system than neutrophils from healthy people, a study has found. This research emphasizes the role of complement in AAV disease development, and suggests the therapeutic potential of its inhibition. The study, titled…
Treatment with Rituxan (rituximab) had some efficacy in eosinophilic granulomatosis with polyangiitis patients, leading to a reduction in glucocorticoid requirement, but asthma and ear, nose and throat (ENT) relapse rates remained high despite continued treatment, a study found. The study, “Efficacy and safety of rituximab in the…
Europe’s umbrella organization for 800 rare disease associations has developed a sweeping initiative to help the continent’s 30 million rare disease patients and their caregivers learn about their conditions, find assistance and receive treatment. Eurordis-Rare Diseases Europe hopes to improve the current piecemeal treatment and support program with a holistic,…
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