Churg-Strauss syndrome

Churg-Strauss syndrome (also known as allergic granulomatosis) is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-heritable, non-transmissible and often mis-diagnosed. Churg-Strauss syndrome was once considered a type of Polyarteritis nodosa (PAN) due to their similar morphologies.

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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 can be difficult, though the increasing use of ANCA assays has made the distinction more routine. Wegener's is closely associated with c-ANCA, unlike Churg-Strauss which shows elevations of p-ANCA.

Disease stages

This disease has three distinct stages.

• The first stage often involves the sinuses and the onset of allergies not previously had or the worsening of pre-existing allergies.
• The second stage involves the onset of acute asthma. Normally, the person would not have had asthma previously.
• The third and final stage involves the various organ systems. Stage three is by far the most life threatening and painful. Often the person will develop severe nerve pain in their legs, arms and hands. Purple marks will appear on the skin and often sores will appear in the mouth or nose. The disease will affect the heart and lungs or it will affect the kidneys and liver.

People can live for many years in the first two stages before progressing to stage three.

Risk stratification

The French Vasculitis Study Group has developed a five-point score ("five-factor score" or FFS) that predicts the risk of death in Churg-Strauss syndrome. These are (1) reduced renal function (creatinine >1.58 mg/dL or 140 μmol/l), (2) proteinuria (>1 g/24h), (3) gastrointestinal hemorrhage, infarction or pancreatitis, (4) involvement of the central nervous system or (5) cardiomyopathy. Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder case (mortality 11.9%).^[1]

Treatment

Treatment for Churg-Strauss syndrome includes glucocorticoids such as prednisone and other immunosupressive 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 life long.

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 less relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.^[2]

Famous patients

The memoir Patient, by the musician Ben Watt (house music producer and half of the band Everything But The Girl), (Grove Press; Reissue edition (September 1998) ISBN 0802135838 ) deals with Watt's mid-1990's experience with Churg-Strauss syndrome, 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 large sections of necrotized intestine, leaving Watt on a permanently restricted diet.

Eponym

It is named for Dr Jacob Churg and Dr Lotte Strauss, who described the condition in 1951.^[3][4]

References

1. ^ Guillevin L, Lhote F, Gayraud M, et al (1996). "Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients". Medicine (Baltimore) 75 (1): 17–28. PMID 8569467.
2. ^ Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA 298 (6): 655–69. doi:10.1001/jama.298.6.655. PMID 17684188.
3. ^ synd/2733 at Who Named It
4. ^ Churg J, Strauss L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am. J. Pathol. 27 (2): 277-301. PMID 14819261.

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