Churg-Strauss vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy). Eosinophilic granulomatosis with polyangiitis (EGPA), also known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy). It usually manifests in three stages. The early (prodromal) stage is marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis. The second stage is characterized by abnormally high numbers of eosinophils (hypereosinophilia), which causes tissue damage, most commonly to the lungs and the digestive tract. The third stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening. This condition is now called 'eosinophilic granulomatosis with polyangiitis' to remove all eponyms from the vasculitides. To facilitate the transition, it was referred to as 'eosinophilic granulomatosis with polyangiitis (Churg–Strauss)' for a period of time starting in 2012. Prior to this it was known as 'Churg–Strauss syndrome', named after Drs. Jacob Churg and Lotte Strauss who, in 1951, first published about the syndrome using the term 'allergic granulomatosis' to describe it. It is a type of systemic necrotizing vasculitis. Effective treatment of EGPA requires suppression of the immune system with medication. This is typically glucocorticoids, followed by other agents such as cyclophosphamide or azathioprine. Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. EGPA is consequently considered a highly variable condition in terms of its presentation and its course. The prodromal stage is characterized by allergy. Almost all patients experience asthma and/or allergic rhinitis, with more than 90% having a history of asthma that is either a new development, or the worsening of pre-existing asthma, which may require systemic corticosteroid treatment. On average, asthma develops from three to nine years before the other signs and symptoms. The allergic rhinitis may produce symptoms such as rhinorrhea and nasal obstruction, and the formation of nasal polyps that require surgical removal, often more than once. Sinusitis may also be present. The second stage is characterized by an abnormally high level of eosinophils (a type of white blood cell) in the blood and tissues. The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract. The signs and symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding. Fever and malaise are often present. The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later. Patients may experience the third stage simultaneously.