Churg-Strauss syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Natural History
Classically, three phases of the disease are described:
Stage 1: The Prodromal Phase
This stage occurs in the teenage years through the 20’s. The first stage often involves the sinuses and the new onset of allergies or the worsening of pre-existing allergies (e.g. atopic diseases, allergic rhinitis).
Stage 2: The Eosinophilic Phase
Peripheral blood eosinophilia is now present. There is eosinophilic infiltration of lung and gastrointestinal (GI) tract. The second stage involves the onset of acute asthma. Normally, the person would not have had asthma previously.
People can live for many years in the first two stages before progressing to stage three.
Stage 3: The Vasculitic Phase
The third phase occurs in the 40’s-50’s and involves multiple organ systems in a life-threatening systemic vasculitis of small and medium sized vessels. 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.
Complications
Complications of Churg-Strauss syndrome depend on the specific organ involved in the disease process.
- Pericarditis
- Myocarditis
- MI
- Heart failure
- Renal failure
- Cerebral hemorrhage
- Gastrointestinal bleeding
- Status asthmaticus
Prognosis
In the past the prognosis of Eosinophilic granulomatosis with polyangiitis was fatal when untreated. Patients who were not treated had an estimated 5 year survival rate of 20 to 30%. Today, the prognosis of Eosinophilic granulomatosis with polyangiitis is heavily dependent on starting treatment with a glucocorticoid.
The following are favorable prognostic factors:
The following are poor prognostic factors:
- These factors are assessed using the Five-Factor Score
- Proteinuria greater than 1g/d
- Creatinine greater than 140 micromoles/l
- Cardiomyopathy
- Gastrointestinal manifestations that are severe
- Central nervous system involvement.
If the Five-Factor Score is 0, 1, or greater than 2 the following mortality rates are as follows: 12%, 26%, and 46%.
- Churg-Strauss syndrome is a serious disease that can be fatal if untreated.
- Untreated patients have a 5 yr survival rate of 20%-30%
Prior to the steroid treatment, the disease was uniformly fatal. Now, 5 year survival tops 70%. In general, poor prognostic indicators include cardiac involvement, GI disease, renal insufficiency (Cr >1.6), proteinuria (>1 g/day) and central nervous system (CNS) involvement. A shorter duration of asthma before the onset of vasculitis also portends poorer prognosis. The role of anti-neutrophil cytoplasmic antibodies (ANCA) in predicting outcome is unclear.
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]