eosinophilia-myalgia syndrome (EMS)

Introduction

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

• generalized myalgias
• fatigue
• muscle weakness
• arthralgias
• edema of the extremities
• skin rash
• oral & vaginal ulcers
• scleroderma-like changes
• ascending neuropathy
• pulmonary manifestations (60%)
  • respiratory distress
  • pulmonary hypertension

Laboratory

• complete blood count (CBC) with differential
  • eosinophilia
• arterial blood gas (ABG) may show progressive hypoxemia

Radiology

• chest x-ray changes may be present
  • diffuse, bilateral reticulonodular infiltrates
  • pleural effusion

Differential diagnosis

• scleroderma or CREST syndrome
• eosinophilic fasciitis
• Spanish toxic oil syndrome (rapeseed oil)

Management

• prednisone
• stop tryptophan supplements (if an issue)

More general terms

• syndrome
• toxicity; poisoning; overdose

Additional terms

• CREST syndrome
• eosinophilic fasciitis (Shulman's syndrome)
• scleroderma (diffuse cutaneous systemic sclerosis)
• toxic oil syndrome
• tryptophan