Churg-Strauss syndrome

Churg-Strauss syndrome mainly involves the medium to small blood vessels of the lungs but eventually also affects the gastrointestinal tract, heart, skin and kidneys.

It may also affect the peripheral nerves. Churg-Strauss syndrome is often referred to as allergic granulomatosis due to its clinical course often starting as a severe form of asthma.

 

About Vasculitis

Vasculitis is a general term referring to a heterogeneous group of diseases that are characterised by inflammatory destruction of blood vessels. The condition occurs if the immune system attacks the blood vessels and this may happen as a result of an infection, a medicine or another disease.

Vasculitis can be acute or chronic and may affect any blood vessel in the body. The inflamed blood vessels cause changes in the vessel wall, including thickening, weakening, narrowing and scarring and this can lead to serious complications damaging the body’s organs. Specific signs and symptoms depend on which organ has been damaged and the extent of the damage. The cause of many forms of vasculitis is poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides (Anti-neutrophil cytoplasmic antibodies).

Example of ANCA-associated vasculitides are granulomatosis with polyangiitis (GPA)  (previously named Wegener's granulomatosis), Churg-Strauss syndrome and microscopic polyangiitis (MPA).

Relevant Literature

  1. Baldini C et al. Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin North Am 2010; 36 : 527-543.
  2. Bosco L et al. Cutaneous manifestations of Churg-Strauss syndrome: report of two cases and review of the literature. Clin Rheumatol 2010; 30: 573-580.
  3. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003; 361: 587-594.
  4. Pagnoux C, Guilpain P, Guillevin L. Churg- Strauss Syndrome. Curr Opinion Rheum 2007; 19: 25-32.