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A. Characteristics [1]navigator

  1. Mean age 43. Male to Female 1.8:1
  2. Very rare disease
  3. Eosinophilic infiltrates into muscle
  4. Likely role for Interleukin 4

B. Symptomsnavigator

  1. Myalgias / Cramping 68%
  2. Swelling in 1 or more extremity 45%
  3. Myopathy / Weakness 15%
  4. Athralgias / Arthritis 10%
  5. Myocarditis / Pericarditis 5%

C. Laboratorynavigator

  1. ESR (erythrocyte sedimentation rate) mildly increased
  2. Aldolase and CPK (muscle function) elevations
  3. Peripheral eosinophilia (>3% of total) ~77%
  4. Eosinophilic Invasion
    1. Causing Direct Myositis 60%
    2. Perimyositis 40%
  5. ANA+ (antinuclear antibodies) 6%
  6. RF+ (rheumatoid factor) 33%

D. Treatment navigator

  1. Glucocorticoids
    1. Prednisone 60mg/day recommended initial dose
    2. ~90% of patients respond
  2. NSAID (indomethacin is usually effective)

E. Differential Diagnosis navigator

  1. Dermatomyositis / Polymyositis
  2. Eosinophilia - myalgia Syndrome
  3. Drug or Toxin Exposure

EOSINOPHILIC FASCIITIS

A. Characteristics navigator
  1. Variant of scleroderma-like disorder; Unknown etiology
  2. Symmetric and painful inflammation with swelling of extremities
  3. Seen in mid 1980s with Tryptophan pill diet supplementation (due to contaminant)

B. Symptoms / Signsnavigator

  1. Usu occurs in middle aged men, precipitated with strenuous activity
  2. Pain, swelling, and inflammation of hands and feet
  3. Induration and thickening of fascia
  4. Fatigue and weight loss are common

C. Therapynavigator

  1. Glucocorticoid: prednisone, 40-60mg per day initially
  2. Rapid taper of prednisone to 5-10mg/day

EOSINOPHILIA-MYALGIA SYNDROME

A. Causenavigator
  1. Epidemiologically associated with ingestion of L-tryptophan (Trp) [5]
  2. "Peak E" impurity (single manufacturer) found to be linked to cases
  3. Chemical structure determined to be unusual dimeric form of L-Trp
  4. Evidence for abnormal metabolism of Trp also found in some patients
  5. Not true autoimmune disease
  6. Increased early death and morbidity from disease; symptoms resolve over time [6]

B. Pathophysiologynavigator

  1. Abnormal tryptophan products stimulate eosinophil activation
  2. Activated eosinophils release toxic proteins including MBP (major basic protein)
  3. Mononuclear cell activation and tissue infiltration seen
  4. Fibrosis of integument in blood vessels, nerves and muscles is observed

C. Presentationnavigator

  1. Myalgias 100%
  2. Skin Rash 71%
  3. Edema 52%
  4. Fever 41%
  5. Arthralgia 35%
  6. Respiratory 32%
  7. Elevated CK, AST

D. Late Complicationsnavigator

  1. Weight Loss 50%
  2. Muscle Weak 44%
  3. Paresthesia 42%
  4. Induration 42%
  5. Alopecia 33%

E. Summary of Clinical Course [2,3,6]navigator

  1. Myalgia and fatigue, usually severe and persistent
  2. Resolution of myalgia in 42% at 1 year; improvement in 72%
  3. Cough and Dyspnea lasted ~13 weeks on average
  4. Diffuse, non-specific rash lasted ~3 months on average
  5. Peripheral paresthesia, numbness or weakness (76%) lasted >12 mo in 33% of patients
  6. Scleroderma-like skin changes persisted in ~25% of patients for >1 year
  7. Therapy [4]
    1. Glucocorticoids appear to have some utility in acute phases of illness
    2. Diphenhydramine, ranitidine, pain and anti-anxiety mediations have some efficacy
  8. Up to 20% will have no resolution at all of severity of disease after 1 year
  9. However, after 18-24 months, nearly all symptoms, except cognitive, are improved [4,6]

EOSINOPHILIC GASTROENTERITIS [7]

A. Introductionnavigator
  1. Rare syndrome of unknown origen
  2. Components
    1. Peripheral eosinophilia
    2. Eosinophilic intiltration of GI tract
    3. Abnormal GI (gastrointestinal) function
  3. Mucosal involvement is most common form
  4. Stomach, duodenum and jejunum are most commonly affected

B. Presentationnavigator

  1. May vary depending on layer of GI tract involved
  2. Postprandial nausea
  3. Vomiting
  4. Abdominal Pain
  5. Diarrhea
  6. Malabsorption
  7. Protein-loss enteropathy
  8. Weight loss

C. Diagnosis and Treatmentnavigator

  1. GI biopsy is required for diagnosis
  2. Random mucosal biopsies are required even when mucosa appears normal
  3. Glucocorticoids are most commonly employed therapy
  4. Patients with allergic history may respond to cromolyn or ketotifen
  5. Surgery may be required for symptoms of obstruction

References navigator

  1. Kaufman LD, Kephart GM, Seidman RJ, et al. 1993. Arthritis Rheum. 36(7):1014 abstract
  2. Culpepper RC, Williams RG, Mease PJ, 1991. Ann Intern Med. 115(6):437 abstract
  3. Varga J, Uitto J, Jimenez SA, et al. 1992. Ann Intern Med. 116(2):140 abstract
  4. Hertzman PA, Clauw DJ, Kaufman LD, et al. 1995. Ann Intern Med. 122(11):851 abstract
  5. Hertzman PA, Blevins WL, Mayer J, et al. 1990. NEJM. 322:860
  6. Sullivan EA, Kamb ML, Jones JL, et al. 1996. Arch Intern Med. 156(9):973 abstract
  7. Galperin C and Gershwin ME. 1997. JAMA. 278(22):1946 abstract