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The Clinical Spectrum of the Eosinophilia-Myalgia Syndrome Associated with L-Tryptophan Ingestion: Clinical Features in 20 Patients and Aspects of Pathophysiology

Richard W. Martin, MD; Joseph Duffy, MD; Andrew G. Engel, MD; J. T. Lie, MD; Carolyn A. Bowles, MD; Thomas P. Moyer, PhD; and Gerald J. Gleich, MD
[+] Article, Author, and Disclosure Information

Grant Support: In part by grants from the National Institutes of Health (NS 6277, AI 15231, AI 09728, and AI 72621), the Council for Responsible Nutrition, and the Mayo Foundation.

Requests for Reprints: Joseph Duffy, MD, Department of Medicine, Mayo Clinic, Rochester, MN 55905.

Current Author Addresses: Drs. Martin, Duffy, Engel, Lie, Bowles, Moyer, and Gleich: Mayo Clinic, Rochester, MN 55905.

© 1990 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1990;113(2):124-134. doi:10.7326/0003-4819-113-2-124
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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 109/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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