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Churg-Strauss Syndrome

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Churg-Strauss Syndrome

Churg-Strauss syndrome
Classification and external resources
10 9 DiseasesDB eMedicine MeSH D015267

Churg–Strauss syndrome (also known as eosinophilic granulomatosis with polyangiitis [EGPA] or allergic granulomatosis[1]) is an autoimmune medium and small vessel vasculitis in persons with a history of airway allergic hypersensitivity (atopy). It usually (but not always) 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 and final stage consists of vasculitis, which can eventually lead to necrosis and is potentially life-threatening.

The syndrome is rare, non-inheritable, and non-transmissible. It was first described by Jacob Churg (1910–2005) and Lotte Strauss (1913–1985) at Mount Sinai Hospital in New York City in 1951.[2][3] It was once considered a type of polyarteritis nodosa due to their similar morphologies.

Historical background

In 1951, pathologists Jacob Churg and Lotte Strauss reported "fever...hypereosinophilia, symptoms of cardiac failure, renal damage, and peripheral neuropathy, resulting from vascular embarrassment in various systems of organs"[4] in a series of thirteen patients with necrotizing vasculitis previously diagnosed as "periarteritis nodosa", accompanied by hypereosinophilia and severe asthma.[5] Churg and Strauss noted three features which distinguished their patients from other patients with periarteritis nodosa but without asthma: necrotizing vasculitis, tissue eosinophilia, and extra vascular granuloma.[5] As a result, they proposed that these cases were evident of a different disease entity, which they referred to as "allergic granulomatosis and angiitis".[5]

Symptoms

Churg-Strauss syndrome consists of three stages; however, not all patients develop all three stages or progress from one stage to the next in an orderly fashion,[6] and 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.[7] Churg-Strauss syndrome is consequently considered a highly variable condition in terms of its presentation and its course.[6][7]

Allergic stage

The prodromal stage is characterized by allergy. Almost all patients experience asthma and/or allergic rhinitis,[8] with more than 90% having a history of asthma that is either a new development, or the worsening of pre-existing asthma[9] which may require systemic corticosteroid treatment.[6] On average, asthma develops from three to nine years before the other signs and symptoms.[6]

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.[8] Sinusitis may also be present.[8]

Eosinophilic stage

The second stage is marked by hypereosinophilia: abnormally high numbers of eosinophils (a type of white blood cell) are found in tissues and in the blood, and cause damage.[6] The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract.[6] The symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding.[6] Fever and malaise are often present.[10]

The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later.[6] Patients may experience the third stage simultaneously.[6]

Vasculitic stage

The third and final stage, and hallmark of Churg-Strauss syndrome is vasculitis: inflammation of blood vessels, and the consequent reduction of blood flow to various organs and tissues.[6] Local and systemic symptoms become more widespread and are compounded by new symptoms from the vasculitis.[10]

Severe complications may arise. Thrombosis of the damaged arteries occurs in severe cases, particularly in the abdominal region, which is followed by infarction and necrosis, or slow atrophy.[10] 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.[10]

The most serious complication of the vasculitic stage is cardiac disease, which is the cause of nearly one half of all deaths in patients with Churg-Strauss syndrome.[10] Among cardiac-related deaths, the most usual cause is eosinophilic myocarditis, although some are due to coronary vasculitis or pericardial tamponade.[10] Renal complications have been reported as being less common.[11]

Diagnosis

Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and anti-neutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from Wegener's granulomatosis is not difficult. Wegener's is closely associated with c-ANCA, while Churg-Strauss can be associated with elevations of p-ANCA.

The American College of Rheumatology 1990 criteria for diagnosis of Churg-Strauss Syndrome lists the following criteria:

  • Asthma
  • Eosinophils greater than 10% of a differential white blood cell count
  • Presence of mononeuropathy or polyneuropathy
  • Non-fixed 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 4 of these 6 criteria are positive. The presence of any 4 or more of the 6 criteria yields a sensitivity of 85% and a specificity of 99.7%.[12]

Risk stratification

The French Vasculitis Study Group has developed a five-point system ("five-factor score" or FFS) that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These five factors are:

The lack of any of these factors indicates milder case, with a five year mortality rate of 11.9%. The presence of 1 factor indicates severe disease, with a five year mortality rate of 26%, and 2 or more very severe disease: 46% 5 year mortality rate.[13]

Treatment

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 that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher, cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.[14]

Montelukast

A case study in 2000 noticed a "marked and severe inflammatory reaction characterized by eosinophilia and glomerulonephritis" and wondered whether this "might have been triggered by the leukotriene receptor antagonist, montelukast."[15] Researchers have searched for links between leukotriene antagonists and Churg–Strauss syndrome; in another study in 2000, researchers did not find a cause-and-effect relationship and wrote: "The occurrence of Churg-Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients."[16]

Famous patients

The memoir Patient, by the musician Ben Watt, deals with Watt's mid-1990s experience with Churg–Strauss syndrome, and his recovery.[17] 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 ten feet of necrotized small intestine, leaving him on a permanently restricted diet.[17]

Umaru Musa Yar'Adua, the president of Nigeria from 2007–2010, reportedly suffered from Churg–Strauss syndrome and died in office of complications of the disease.[18]

The DJ and author Charlie Gillett was diagnosed with Churg-Strauss in 2006; he died four years later.[19]

References

Bibliography

External links

  • Churg-Strauss syndrome – MedLink Neurology Clinical Summary
  • GPnotebook
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