Rheumatology Book


Eosinophilia-Myalgia Syndrome

Aka: Eosinophilia-Myalgia Syndrome
  1. See Also
    1. Fibromyalgia
    2. Eosinophilia
    3. Hyperesoinophilic Syndrome
    4. Myofascial Pain Syndrome
  2. Epidemiology
    1. Rare Condition associated with an unknown contaminant L-Tryptophan supplements from a single Tokyo manufacturer
      1. However case reports continue with L-Tryptophan and 5-Hydroxytryptophan (5-HTP) supplement use
    2. Ultimately >1500 confirmed cases (actual numbers estimated at 5,000-10,000) and resulted in 37 deaths
    3. Gender: Women were most often affected
    4. U.S. had most reported cases, but
  3. History
    1. First described in 1989 in New Mexico and Minnesota
    2. Associated with L-Tryptophan and 5-Hydroxytryptophan (5-HTP) supplement use
    3. Resulted in a U.S. recall of L-Tryptophan supplements in 1989
  4. Associated Conditions
    1. Hypothyroidism
  5. Pathophysiology
    1. Linked to L-Tryptophan and 5-Hydroxytryptophan (5-HTP) usage
    2. Suspected cause is an unknown contaminant in the supplements
  6. Symptoms
    1. Myalgias (Uniformly present)
      1. Rapid onset with persistent pain
      2. Severe and incapacitating pain (limits Activities of Daily Living)
      3. Associated with Muscle spasms (esp. calves, Abdomen)
      4. Associated with perceived weakness
      5. Exacerbated by movement
    2. Arthralgias (73%)
    3. Pruritus
    4. Non-productive cough with Dyspnea (59%)
    5. Neuropathy (27%)
      1. Paresthesia or Hypesthesia (e.g. Burning Sensation)
  7. Signs
    1. Rash (60%)
    2. Peripheral Edema (59%)
      1. Cutaneous non-Pitting Edema
    3. Periorbital edema (28%)
    4. Fever (36%)
    5. Alopecia (28%)
    6. Hepatomegaly (5%)
    7. Thickened skin (32%)
  8. Labs
    1. Complete Blood Count (CBC)
      1. Elevated Peripheral Eosinophil Count
      2. Leukocytosis (85%)
    2. Aldolase increase (46%)
    3. Creatine Kinase increase (10%)
    4. Erythrocyte Sedimentation Rate (ESR) increased (33%)
    5. Liver Function Test (LFT) abnormality (43%)
    6. Antinuclear Antibody (ANA) frequently positive
      1. Speckled pattern most common
  9. Radiology: Chest XRay findings
    1. Infiltrates (17%)
    2. Pleural Effusions (12%)
  10. Differential Diagnosis
    1. See Eosinophilia
    2. Polymyositis
    3. Polymyalgia Rheumatica
    4. Trichinosis
    5. Polyarteritis Nodosa
    6. Churg Strauss
    7. Systemic Sclerosis
    8. Hypereosinophilic Syndrome
  11. Diagnosis: Criteria from CDC Cases
    1. Peripheral Eosinophil Count >1000 cells/mm
    2. Severe myalgias that interfere with daily activity
    3. Exclude other diagnoses
      1. Infection
      2. Neoplastic Disease
      3. Other diagnosis responsible
  12. Management
    1. General Therapies
      1. Systemic Corticosteroids
        1. Not uniformly effective
        2. Unlikely effective if no response by 4-6 weeks
        3. Predictors of response
          1. Edema
          2. Eosinophilia
        4. High doses are required initially
      2. Other experimental therapies
        1. Methotrexate
        2. Vitamin B6
        3. Cyclosporine
        4. Cyclophosphamide
        5. Plasmapheresis
    2. Myalgias and Muscle spasms (very difficult to treat)
      1. Quinine sulfate
      2. Tricyclic Antidepressants
      3. Methocarbamol
      4. Cyclobenzaprine
      5. Dantrolene
      6. Baclofen
      7. Benzodiazepines
      8. Narcotics Analgesics may be needed
      9. Chronic Pain Management
  13. Resources
    1. Eosinophilia-Myalgia Syndrome (NIH)
      1. https://rarediseases.org/rare-diseases/eosinophilia-myalgia-syndrome/
  14. Reference
    1. Freundlich in Kelley (1993), Textbook Rheumatology, Saunders, Philadelphia, p. 1150-7
    2. Hertzman (1995) J Rheumatol 22(1):161-3 [PubMed]
    3. Swygart (1990) JAMA 264:1698-1703 [PubMed]

Eosinophilia-Myalgia Syndrome (C0085179)

Definition (MSH) A complex systemic syndrome with inflammatory and autoimmune components that affect the skin, fascia, muscle, nerve, blood vessels, lung, and heart. Diagnostic features generally include EOSINOPHILIA, myalgia severe enough to limit usual activities of daily living, and the absence of coexisting infectious, autoimmune or other conditions that may induce eosinophilia. Biopsy of affected tissue reveals a microangiopathy associated with diffuse inflammation involving connective tissue. (From Spitzer et al., J Rheumatol Suppl 1996 Oct;46:73-9; Blackburn WD, Semin Arthritis Rheum 1997 Jun;26(6):788-93)
Concepts Disease or Syndrome (T047)
MSH D016603
ICD9 710.5
SnomedCT 95416007
English Eosinophilia Myalgia Syndrome, Eosinophilia-Myalgia Syndromes, Syndrome, Eosinophilia-Myalgia, Syndromes, Eosinophilia-Myalgia, EMS - Eosinophil myalgia syndr, Eosinophilia-Myalgia Syndrome, eosinophilia-myalgia syndrome, eosinophilia-myalgia syndrome (diagnosis), Eosinophilia myalgia snd, Eosinophilia-Myalgia Syndrome [Disease/Finding], eosinophilia myalgia syndrome, Eosinophilia-myalgia syndrome, EMS - Eosinophilia myalgia syndrome, Eosinophilia myalgia syndrome, Eosinophilia myalgia syndrome (disorder), eosinophilia; myalgia syndrome, syndrome; eosinophilia-myalgia
Italian Sindrome eosinofilia e mialgia, Sindrome eosinofilia-mialgia
Swedish Eosinofili-myalgisyndrom
Czech syndrom myalgický s eozinofilií, Syndrom myalgie s eozinofilií
Finnish Eosinofilia-myalgiaoireyhtymä
Japanese 好酸球増加症-筋肉痛症候群, 好酸球増加筋痛症候群, コウサンキュウゾウカキンツウショウコウグン, 好酸球増加・筋痛症候群, 好酸球増多筋痛症候群, 好酸球増多筋肉痛症候群
Polish Zespół eozynofilia-mialgia, Eozynofilia i mialgia w reakcji na tryptofan
Hungarian eosinophilia myalgia syndroma
Norwegian Eosinofili-myalgisyndrom
Dutch eosinofilie; myalgie-syndroom, syndroom; eosinofilie-myalgie, eosonofilie-myalgiasyndroom, EMS, Eosinofilie-myalgiesyndroom (EMS), Syndroom, eosinofilie-myalgie- (EMS)
Spanish síndrome de mialgia eosinofílica, síndrome de mialgia eosinófila (trastorno), síndrome de mialgia eosinófila, Síndrome de eosinofilia-mialgia, Síndrome de Eosinofilia-Mialgia
Portuguese Síndrome eosinofilia-mialgia, Síndrome de Eosinofilia-Mialgia
French Syndrome éosinophilie-myalgies, Syndrome éosinophilie-myalgie
German Eosinophilie-Myalgie-Syndrom
Derived from the NIH UMLS (Unified Medical Language System)

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