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7 de feb. de 2024 · Overview. Churg-Strauss syndrome is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them. This condition is also known as eosinophilic granulomatosis with polyangiitis (EGPA). Adult-onset asthma is the most common sign of Churg-Strauss syndrome.
Asthma is one of the cardinal features of EGPA. Asthma symptoms may begin long before the onset of vasculitis – e.g., many years before any other symptoms of EGPA arise, and long before the diagnosis of EGPA is made. Other early symptoms/signs include nasal polyps and allergic rhinitis.
Asthma may precede the development of systemic vasculitis by up to 30 years; however, a shorter duration of asthma before the onset of vasculitis is associated with a poorer prognosis (a mean interval of 6.6 years between onset of asthma and vasculitis).
22 de abr. de 2014 · For many years, the contribution of vasculature to severe asthma was limited to discussion of small and medium vessel vasculitis commonly referred to as Churg − Strauss syndrome. This comprehensive review will explore the known mechanisms that are associated with remodeling of the vasculature in a variety of critical asthma presentations.
9 de may. de 2023 · Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and...
23 de mar. de 2012 · The pulmonary vasculitides are a rare group of heterogeneous disorders unified by the histopathologic finding of inflammation and destruction of the blood vessel wall.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis comprises three syndromes, all with frequent respiratory manifestations. Studies indicate that ANCA specificity is more important for prognosis, relapse risk, response to therapy and outcomes than the specific diagnosis.
