Churg-Strauss Syndrome Results in Fatality Due to Missed Diagnosis

This case involves a twenty-eight-year-old woman who was admitted to a hospital with a chief complaint of a fever of unknown origin. The patient had a history of bronchial asthma and chronic bronchitis that did not respond to multiple courses of antimicrobial treatment including amoxicillin, clavulanic acid, moxifloxacin hydrochloride, cefotaxime, and levofloxacin. The patient was worked up for several respiratory conditions including tuberculosis but all tests came back negative. Two weeks into her admission and after several rounds of diagnostic testing, the patient sustained a massive heart attack. CPR and resuscitative measures were instituted but the patient died after forty-five minutes of compression without a return of a normal heart rhythm. Autopsy reports showed that the cause of her illness was Churg-Strauss syndrome (CSS), which is a known cause of a fever of unknown origin.

Question(s) For Expert Witness

1. What are the most common manifestations of CSS?

Expert Witness Response E-000738

Churg-Strauss syndrome is a granulomatous small-vessel vasculitis. The cause is unknown. CSS is significantly less common than the other forms of ANCA-associated vasculitis. The annual incidence of CSS is approximately 2.4 cases per hundred thousand individuals, with a roughly equal distribution of cases between men and women. The clinical features of CSS include asthma, eosinophilia greater than 10% in the peripheral blood, evidence of a neuropathy in a vasculitic pattern (eg, mononeuritis multiplex) or polyneuropathy, pulmonary infiltrates that may be transient, a history of sinus disease, and evidence of extravascular eosinophilia on biopsy. Most deaths in cases of CSS result from cardiac involvement, which is seen in 80% of cases. The manifestations of CSS cardiac involvement include cardiac arrest, myocardial infarction, valvular heart disease, congestive heart failure, pericardial effusion, and constrictive pericarditis.