Search
eosinophilia-myalgia syndrome (EMS)
Multisystemic inflammatory disorder with eosinophilia & myalgias.
Etiology:
-> epidemic in 1989 linked to ingestion of L-tryptophan nutritional supplements. Uncertainty may still exist as to the exact association: whether it was L-tryptophan itself, an impurity in the supplements identified by analytical chromatography *, or if tryptophan intake was excessive.
* It was found that one manufacturer supplied the majority of the L-tryptophan associated with EMS and there were certain changes and deficiencies in their manufacturing procedures.
Possible contaminants include 1,1'-ethylidenebis(tryptophan) and 3-(phenyl amino)alanine.
Clinical manifestations:
1) generalized myalgias
2) fatigue
3) muscle weakness
4) arthralgias
5) edema of the extremities
6) skin rash
7) oral & vaginal ulcers
8) scleroderma-like changes
9) ascending neuropathy
10) pulmonary manifestations (60%)
a) respiratory distress
b) pulmonary hypertension
Laboratory:
1) complete blood count (CBC) with differential
- eosinophilia
2) arterial blood gas (ABG) may show progressive hypoxemia
Radiology:
- chest x-ray changes may be present
a) diffuse, bilateral reticulonodular infiltrates
b) pleural effusion
Differential diagnosis:
1) scleroderma or CREST syndrome
2) eosinophilic fasciitis
3) Spanish toxic oil syndrome (rapeseed oil)
Management:
1) prednisone
2) stop tryptophan supplements (if an issue)
Related
CREST syndrome
eosinophilic fasciitis (Shulman's syndrome)
scleroderma (diffuse cutaneous systemic sclerosis)
toxic oil syndrome
tryptophan
General
syndrome
toxicity; poisoning; overdose
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 783-84
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
- Das et al. Toxicology Letters 150:111-122, 2004