Objective: To describe some of the most severe features of eosinophilia-myalgia syndrome (EMS) and identify potential prognostic indicators.
Methods: Systematic review of data from initial case reports and from followup supplemental death report forms forwarded to the national surveillance system administered by the US Centers for Disease Control (CDC).
Results: As of August 10, 1991 36 deaths related to EMS had been reported to CDC. Among all patients fitting the surveillance case definition for EMS, we found that patients who died were older, had higher absolute leukocyte and eosinophil counts, and reported a greater frequency of cough or dyspnea, neuropathy, hepatomegaly, leukocytosis, and elevated erythrocyte sedimentation rate. All patients who died had illnesses affecting multiple organ systems. Of the 36 patients who died, 33 (92%) had neuromuscular sequelae, 29 (81%) had pulmonary complications, and 23 (64%) had cardiac manifestations. The most commonly observed disease process leading to death was progressive polyneuropathy and myopathy (24 of the 36 reported deaths) which produced complications of pneumonia and sepsis or respiratory failure due to weakness; cardiomyopathy was the underlying cause of death for 4 patients, primary pulmonary disease for 3, sudden death attributed to arrhythmia for 2, stroke for 2, and septic complications of therapy for one.
Conclusion: Although neuromuscular complications were the most prominent sequelae among patients reported to have died, this is clearly a multisystemic disease. Older age and involvement of more than one organ system suggest a particularly poor prognosis, and the neuromuscular, pulmonary and cardiovascular sequelae appear to be the most worrisome.