Some symptoms of the eosinophilia-myalgia syndrome were unresolved at 1 year
ACP J Club. 1992 Jan-Feb;116:25. doi:10.7326/ACPJC-1992-116-1-025
Culpepper RC, Williams RG, Mease PJ, Koepsell TD, Kobayashi JM. Natural history of the eosinophilia-myalgia syndrome. Ann Intern Med. 1991 Sep 15;115:437-42.
To describe the 1-year prognosis for patients with the eosinophilia-myalgia syndrome.
An inception cohort with the syndrome was identified and followed by means of telephone interviews and examinations.
Patients were reported to the Washington State Department of Health by their physicians.
47 patients had onset of symptoms between 1 July and 12 December 1989. Diagnosis of the eosinophilia-myalgia syndrome was made according to the Centers for Disease Control surveillance case definition. This included an eosinophil count greater than 1 × 109/L, generalized myalgia severe enough to affect activities of daily living, and absence of infection or neoplasm. Patients were mostly white (98%); 87% were women, and 56% were aged 35 to 59 years. All patients reported use of products containing L-tryptophan. 45 patients (95%) were followed for a mean of 14 months.
Assessment of prognostic factors
Data on patient demographics, symptoms, laboratory results, diagnostic findings, and treatment regimens were obtained from the referring physician, from a series of 4 telephone interviews with the patients, and from examination of 15 patients.
Main outcome measures
The patients' assessment of symptom onset, severity, duration, and resolution, confirmed by examinations by the referring physician or the investigators. Severity and duration of symptoms in patients taking prednisone were compared with those of the remainder of the cohort.
At diagnosis all patients reported myalgia and fatigue, which were generally severe and persistent. Myalgia had resolved in 42% of patients by 1 year and improved by an average of 72% in the others. Cough or dyspnea (80%) lasted for 13 weeks (range 1 to 65 weeks). 11 patients developed interstitial lung disease, and 3 developed pulmonary hypertension. Diffuse, nonspecific rash (80%) lasted for a mean of 3 months. Peripheral edema (93%) was reported to be improved by prednisone or furosemide and lasted a median of 27 weeks. Peripheral paresthesia, numbness, or weakness (76%) was still present in 16 patients 12 months after onset. Scleroderma-like skin changes (42%) persisted in 12 patients. 7 patients overall had less than 50% improvement from their most severe stage of illness. Treatment with prednisone (28 patients) made no difference to the course of the disease.
Myalgia, fatigue and scleroderma-like skin changes persisted in more than 50% of patients with the eosinophilia-myalgia syndrome after 1 year.
Source of funding: Not stated.
Address for article reprint: Dr. R.C. Culpepper, Great Lakes Naval Hospital, OHPMD Code 037, Great Lakes, IL 60088, USA.
Physicians who see patients with the diagnosis of the eosinophilia-myalgia syndrome will be interested in this 1-year follow-up of patients. Physicians who do not work in this field should not rest too comfortably. They may see new patients in whom the eosinophilia-myalgia syndrome has not previously been diagnosed. They may also see patients with this condition who develop sequelae not immediately recognizable as part of the syndrome.
What do these patients present with (1)? Most patients have important chronic manifestations such as fatigue, myalgia, and scleroderma-like changes of the skin (limbs, neck, face). Patients with pulmonary abnormalities (coughing, dyspnea) and late central nervous system neurocognitive changes could present a difficult diagnostic problem if a history of use of products containing L-tryptophan is not obtained. Chronic muscle cramping in the extremities or intermittent flares of myalgia can occur, especially after exercise.
Can the patient at risk for long-term sequelae be recognized early in the disease process? No predictors have yet been determined (2).
Is corticosteroid therapy helpful? Some authors recommend corticosteroids in the management of acute disease. The results of this study suggest that disease duration was not affected by prednisone, yet patients reported that it was the "most effective treatment." Patients with interstitial pulmonary disease and pulmonary hypertension reported subjective improvement with steroids. However, as in all other "hypersensitivity" reactions to drugs (e.g., to phenytoin) or contaminants (e.g., the toxic oil syndrome), the role of corticosteroids for the eosinophilia-myalgia syndrome associated with L-tryptophan is controversial. There are no randomized trials to answer the question.
Neil H. Shear, MD
Sunnybrook and Women's College Health Sciences CentreToronto, Ontario, Canada
Neil H. Shear, MD
Sunnybrook and Women's College Health Sciences Centre
Toronto, Ontario, Canada