1990 criteria for the classification of Churg-Strauss Syndrome, their sensitivity and specificity versus other defined vasculitis syndromes*.

NB: Please see our page:  Important Information

*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%.

• Asthma
• Eosinophilia >10%
• Neuropathy, mono or poly
• Pulmonary infiltrates, non-fixed
• Paranasal sinus abnormality
• Extravascular eosinophils

Eosinophilic vasculitis:

Churg-Strauss is an example. Eosinophils are predominant.

Granulomatous vasculitis:

Wegeners Granulomatosis and Churg-Strauss Angiitis. Cells include histiocytes, plasma cells, lymphocytes and occasional giant cells.

Giant cells arteritis:

Are found in cranial and temporal arteritis and polymyalgia rheumatica. Multinucleated giant cells are predominant.

The optimum specimen selected for biopsy should be an early established lesion about 18-36 hours old.

The traditional view that Sweet Syndrome is a form of vasculitis is now being challenged. Many dermatopathologist no longer consider this condition a true vasculitis, because the histopathology merely consists of dense dermal perivascular neutrophilic aggregates, without fibrinoid necrosis of the vessel wall.

Churg-Strauss (Granulomatosis) Angiitis

has features of a necrotising granulomatosus and eosinophilic vasculitis.

Classic Polyarteritis Nodosa (PAN)

This is a multisystem, narcotising vasculitis of small and medium sized muscular arteries with characteristic involvement of renal and visceral arteries. 30% are HBsAg +ve. The mean age of onset is 45 years with a male preponderance (M/F 2.5 to 1). Systemic features include fever, malaise, arthralgia, myalgia, abdominal pain, melaena, CVA, neuropathy, hypertension and renal failure. Cutaneous lesions occur in 50% of cases with painful subcutaneous nodules, purpura, ulcers, infarct and gangrene along the course of arteries and a livedo reticularis pattern. Microscopic PAN is a variant with small vessel vasculitis and focal segmented glomerulonephritis.

Investigations include skin biopsy of nodular lesions, sural nerve biopsy and nerve conduction study. Blood test showed raised ESR, leucocytosis with neutophilia, anaemia of chronic disease, hypergammaglobulinaemia, and positive Hep B surface antigen. Renal and visceral artery involvement are demonstrated by small aneurysmal dilatations on visceral angiography. Classic PAN does not involve pulmonary arteries; granuloma and significant eosinophilia are not part of the syndrome.

Treatment and course:

The clinical course is characterised by progressive deterioration with intermittent acute flare ups. Death usually results from renal failure. The 5-year survival of untreated patients is 13%, but with combined systemic steroid and cyclophosphamide therapy there may be a 90% long-term remission rate even after discontinuing therapy.

Cutaneous Polyarteritis Nodosa

A more benign form PAN where vasculitis is chiefly confined to the skin only. Cutaneous manifestation is similar but milder than classic PAN; nodules and livedo, with accompanying fever, arthralgia and neuropathy occasionally.

Treatment includes low dose systemic steroids. The prognosis is good with no significant mortality even without treatment.

Churg-Strauss (Allergic Granulomatous) Angiitis

Churg-Strauss is a systemic vasculitis which shares many features with classic PAN, but distinguished by the following features :

(i) Frequent involvement of the lung.

(ii) Vasculitis of blood vessels of various types and sizes.

(iii) Intravascular and extravascular granuloma formation with eosinophil infiltration.

(iv) Strong association with severe asthma and peripheral eosinophilia.

(v) Skin lesions occur in 70% of cases but renal disease is less common or severe as PAN.

The 5-year survival of untreated Churg-Strauss is 25%. The cause of death is often related to cardio-pulmonary involvement as opposed to renal and gastro-intestinal involvement of PAN.

Glucocorticoid therapy alone improves 5 year survival to 50%. Combination regimen of cyclophosphamide and alternate day prednisolone is required for more severe cases and may result in high rate of remission approaching that of classic PAN.

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