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, Joseph Duffy, Andrew G Engel, J. T. Lie, Carolyn A. Bowles, Thomas P. Moyer, Gerald J. Gleich

Research output: Contribution to journalArticle

121 Citations (Scopus)

Abstract

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 × 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 eosinophilderived 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.

Original languageEnglish (US)
Pages (from-to)124-134
Number of pages11
JournalAnnals of Internal Medicine
Volume113
Issue number2
StatePublished - Jul 15 1990

Fingerprint

Eosinophilia-Myalgia Syndrome
Tryptophan
Eating
Eosinophils
Poisons
Myalgia
Eosinophilia
Eosinophil Granule Proteins
Proteins
Viscera
Fascia
Myocarditis
Neurotoxins
Common Bile Duct
Brain Diseases
Exudates and Transudates
Centers for Disease Control and Prevention (U.S.)
Diuretics
Respiratory Insufficiency
Fluorescent Antibody Technique

ASJC Scopus subject areas

  • Medicine(all)

Cite this

The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion : Clinical features in 20 patients and aspects of pathophysiology. / Martin, Richard W.; Duffy, Joseph; Engel, Andrew G; Lie, J. T.; Bowles, Carolyn A.; Moyer, Thomas P.; Gleich, Gerald J.

In: Annals of Internal Medicine, Vol. 113, No. 2, 15.07.1990, p. 124-134.

Research output: Contribution to journalArticle

Martin, Richard W. ; Duffy, Joseph ; Engel, Andrew G ; Lie, J. T. ; Bowles, Carolyn A. ; Moyer, Thomas P. ; Gleich, Gerald J. / The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion : Clinical features in 20 patients and aspects of pathophysiology. In: Annals of Internal Medicine. 1990 ; Vol. 113, No. 2. pp. 124-134.
@article{37d983df6f6d4f9d8bc753441719ae60,
title = "The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion: Clinical features in 20 patients and aspects of pathophysiology",
abstract = "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 × 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 eosinophilderived 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.",
author = "Martin, {Richard W.} and Joseph Duffy and Engel, {Andrew G} and Lie, {J. T.} and Bowles, {Carolyn A.} and Moyer, {Thomas P.} and Gleich, {Gerald J.}",
year = "1990",
month = "7",
day = "15",
language = "English (US)",
volume = "113",
pages = "124--134",
journal = "Annals of Internal Medicine",
issn = "0003-4819",
publisher = "American College of Physicians",
number = "2",

}

TY - JOUR

T1 - The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion

T2 - Clinical features in 20 patients and aspects of pathophysiology

AU - Martin, Richard W.

AU - Duffy, Joseph

AU - Engel, Andrew G

AU - Lie, J. T.

AU - Bowles, Carolyn A.

AU - Moyer, Thomas P.

AU - Gleich, Gerald J.

PY - 1990/7/15

Y1 - 1990/7/15

N2 - 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 × 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 eosinophilderived 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.

AB - 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 × 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 eosinophilderived 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.

UR - http://www.scopus.com/inward/record.url?scp=0025315815&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025315815&partnerID=8YFLogxK

M3 - Article

C2 - 2360751

AN - SCOPUS:0025315815

VL - 113

SP - 124

EP - 134

JO - Annals of Internal Medicine

JF - Annals of Internal Medicine

SN - 0003-4819

IS - 2

ER -