The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion. Clinical features in 20 patients and aspects of pathophysiology.
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 greater than 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 less than 0.01 and P less than 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.
Martin
R W
RW
Mayo Clinic and Foundation, Rochester, Minnesota.
Duffy
J
J
Engel
A G
AG
Lie
J T
JT
Bowles
C A
CA
Moyer
T P
TP
Gleich
G J
GJ
D000328:Adult; D000368:Aged; D004802:Eosinophilia / Q000139:chemically induced* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D005208:Fasciitis / Q000139:chemically induced; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000139:chemically induced; D007249:Inflammation / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000139:chemically induced* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D009205:Myocarditis / Q000139:chemically induced; D009422:Nervous System Diseases / Q000139:chemically induced; D010146:Pain / Q000139:chemically induced* / Q000503:physiopathology; D059408:Pain Management; D011658:Pulmonary Fibrosis / Q000139:chemically induced; D014364:Tryptophan / Q000009:adverse effects* / Q000032:analysis
D014364:Tryptophan