Asthma Plus Numbness and Joint Pain: Don't Miss This Diagnosis

Stephen Paget, MD

Disclosures

March 07, 2012

Key Points for Practice

This case raises many interesting and important points regarding Churg-Strauss vasculitis, including the following:

  1. ANCAs are not found in more than 50% of patients with Churg-Strauss vasculitis. In 2005, a multicenter study in Annals of Internal Medicine[1] reported the results of 112 patients with Churg-Strauss vasculitis from 63 clinical centers in France, Belgium, Latvia, and the United Kingdom. The authors detected ANCA in 43 (38%) patients. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever.

  2. Cardiac abnormalities may be a prominent manifestation of Churg-Strauss vasculitis. Guillevin and coworkers[2] identified myocardial involvement (which was associated with a poor prognosis) in 14% of patients in this large series. Churg-Straus disease should be considered in adults with asthma and eosinophilia who develop chest pain, shortness of breath, and cardiogenic shock. Cardiac evaluation may demonstrate eosinophilic pericarditis, cardiomegaly, restrictive cardiomyopathy-perimyocarditis, diminished myocardial contractility due to myocardial or endocardial eosinophilic vasculitis, or associated tissue infarction. These changes and pericardial effusion are important prognostic factors that obviously bear on the degree and time course of Churg-Strauss-associated heart failure. Both systolic and diastolic dysfunction may be found. A steady decline in myocardial shortening fraction often follows. Heart failure is an important cause of death in Churg-Strauss disease and is a major determinant of prognosis. In the series by Guillevin and associates, 8% of patients with the condition died of cardiac disease in the acute phase of the illness.

  3. Although some patients with Churg-Strauss vasculitis may be treated solely with high doses of corticosteroids, many patients with signs associated with a poor prognosis also need to be treated with immunosuppressive drugs, such as azathioprine, mycophenolate, or cyclophosphamide. The French Vasculitis Study Group[3] has developed a 5-point score ("five-factor score," or FFS) that predicts the risk for death in Churg–Strauss syndrome. They are 1) reduced renal function (creatinine levels >1.58 mg/dL or 140 μmol/L); 2) proteinuria (>1 g/24 hr); 3) gastrointestinal hemorrhage, infarction, or pancreatitis; 4) involvement of the central nervous system; or 5) cardiomyopathy. The presence of one of these factors indicates severe disease (5-year mortality, 26%), and 2 or more indicate very severe disease (5-year mortality, 46%); absence of all 5 indicates a milder case (5-year mortality, 11.9%).

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