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Churg–Strauss syndrome (CSS, also known as eosinophilic granulomatosis with polyangiitis [EGPA] or allergic granulomatosis) is an 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.
Effective treatment of Churg–Strauss syndrome requires suppression of the immune system with medication. This is typically glucocorticoids followed by other agents such as cyclophosphamide or azathioprine.
Signs and symptoms
Churg–Strauss syndrome 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. Churg–Strauss syndrome 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 third and final stage, and hallmark of Churg–Strauss syndrome, is inflammation of the blood vessels, and the consequent reduction of blood flow to various organs and tissues. Local and systemic symptoms become more widespread and are compounded by new symptoms from the vasculitis.
Severe complications may arise. Blood clots may develop within the damaged arteries in severe cases, particularly in arteries of the abdominal region, which is followed by infarction and cell death, or slow atrophy. Many patients experience severe abdominal complaints; these are most often due to peritonitis and/or ulcerations and perforations of the gastrointestinal tract, but occasionally due to acalculous cholecystitis or granulomatous appendicitis.
The most serious complication of the vasculitic stage is heart disease, which is the cause of nearly one-half of all deaths in patients with Churg–Strauss syndrome. Among heart disease-related deaths, the most usual cause is inflammation of the heart muscle caused by the high level of eosinophils, although some are deaths to inflammation of the arteries that supply blood to the heart or pericardial tamponade. Kidney complications have been reported as being less common.
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and antineutrophil cytoplasmic antibodies against neutrophil granulocytes. The American College of Rheumatology 1990 criteria for diagnosis of Churg–Strauss syndrome lists these criteria:
- Eosinophils greater than 10% of a differential white blood cell count
- Presence of mononeuropathy or polyneuropathy
- Unfixed pulmonary infiltrates
- Presence of paranasal sinus abnormalities
- Histological evidence of extravascular eosinophils
For classification purposes, a patient shall be said to have Churg–Strauss syndrome (CSS) if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%.
The French Vasculitis Study Group has developed a five-point system ("five-factor score") that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These factors are:
- Reduced renal function (creatinine >1.58 mg/dl or 140 μmol/l)
- Proteinuria (>1 g/24h)
- Gastrointestinal hemorrhage, infarction, or pancreatitis
- Involvement of the central nervous system
The lack of any of these factors indicates milder case, with a five-year mortality rate of 11.9%. The presence of one factor indicates severe disease, with a five-year mortality rate of 26%, and two or more indicate very severe disease: 46% five-year mortality rate.
Treatment for Churg–Strauss syndrome includes glucocorticoids (such as prednisolone) and other immunosuppressive drugs (such as azathioprine and cyclophosphamide). In many cases, the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and lifelong.
A systematic review conducted in 2007 indicated all patients should be treated with high-dose steroids, but in patients with a five-factor score of one or higher, cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than six. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.
Churgh-Strauss syndrome was first described by pathologists Jacob Churg (1910–2005) and Lotte Strauss (1913–1985) at Mount Sinai Hospital in New York City in 1951. They reported "fever...hypereosinophilia, symptoms of cardiac failure, renal damage, and peripheral neuropathy, resulting from vascular embarrassment in various systems of organs" in a series of 13 patients with necrotizing vasculitis previously diagnosed as "periarteritis nodosa", accompanied by hypereosinophilia and severe asthma. Churg and Strauss noted three features which distinguished their patients from other patients with periarteritis nodosa but without asthma: necrotizing vasculitis, tissue eosinophilia, and extravascular granuloma. As a result, they proposed that these cases were evident of a different disease entity, which they referred to as "allergic granulomatosis and angiitis".
The memoir Patient, by musician Ben Watt, deals with his experience with Churg–Strauss syndrome in 1992, and his recovery. Watt's case was unusual in that it mainly affected his gastrointestinal tract, leaving his lungs largely unaffected; this unusual presentation contributed to a delay in proper diagnosis. His treatment required the removal of 15 ft of necrotized small intestine (about 75%), leaving him on a permanently restricted diet.
Japanese ski jumper Taku Takeuchi, who won the bronze medal in the team competition, suffers from the disease and competed at the Sochi Olympics less than a month after being released from hospital treatment.
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