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We describe the clinical spectrum of the L-tryptophan-associated eosinophilia-myalgia syndrome in 20 patients.In all but one case,patients met the Centers for Disease Control(CDC)case definition for the syndrome: peripheral blood eosinophilia(eosinophil count>1.0 x 10 9 /L)and generalized,disabling myalgias without other recognized causes.Three patients with eosinophilia and myalgia developed eosinophilic fasciitis, and 4 other patients developed,respectively,pneumonitis and myocarditis, neuropathy culminating in respiratory failure,encephalopathy,and fibrosis about the common bile duct.No relation was apparent between dose or duration of L-tryptophan exposure and the eosinophilia-myalgia syndrome.No organic contaminants were identified in L-tryptophan preparations taken by patients or asymptomatic users when these preparations were examined by chromatography or mass spectroscopy.Biopsy specimens in 12 patients showed a mononuclear exudate with a variable admixture of eosinophils in affected tissues,including skin,fascia,muscle,and some viscera.Eosinophil toxic granule proteins,major basic protein,and eosinophil-derived neurotoxin were elevated in the serum and urine of patients compared with normal control subjects(P<0.01 and P<0.02,respectively).Immunofluorescence showed major basic protein deposited outside of eosinophils in affected tissues, indicating that toxic granule proteins are released in diseased organs. Treatment included withdrawal of L-tryptophan in all cases.Corticosteroids were prescribed for 16 patients and diuretics alone for 1 patient;no drugs were prescribed for 3 patients.Four patients have recovered fully,others are stable or slowly recovering,and 1 is gravely ill despite prolonged treatment. |
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