Churg-Strauss syndrome

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**Churg-Strauss syndrome**
*Classification & external resources*
ICD-10	M 30.1
ICD-9	446.4
DiseasesDB	2685
eMedicine	med/2926

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. Churg-Strauss syndrome is classified both as a type of polyarteritis nodosa and, more broadly, as a diffuse connective tissue disease. It is a rare disease that is non-heritable, non-transmissale and often mis-diagnosed.

1 Diagnosis
2 Disease stages
3 Treatment
4 Famous patients
5 External links

[edit] Diagnosis

Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and pANCA antibodies against neutrophil granulocytes. Differentiation from Wegener's granulomatosis can be difficult.

[edit] 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.

[edit] Treatment

Treatment for Churg-Strauss syndrome includes glucocorticoids such as Prednisone and other immunosupressive drugs such as Imuran and Cytoxinor. 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.

[edit] 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.