Graves' ophthalmopathy E05.0 H06.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

endocrine ophthalmopathy; Endocrine orbitopathy; EO; Exophthalmos; Graves' ophthalmopathy; Graves' orbitopathy; Ophthalmopathy edocrine; orbitopathy endocrine

Definition
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Inflammatory disease of the orbit characterized by an increasing exophthalmus. The disease belongs to the organ-specific autoimmune diseases and represents in > 90% of the cases a partial manifestation of an immunogenic hyperthyroidism (M.Basedow).

Etiopathogenesis
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Ultimately unknown. A genetically induced autoimmune disease is assumed(autoantibodies against TSH receptor = TRAK). The pathogenetic relation to orbital tissue results from the presence of TSH receptors. There is an infiltration of autoreactive T lymphocytes, a reactive fibroblast proliferation and an accumulation of gykosaminoglycans (GAG) in the periorbital tissue (exophthalmus) and the eye muscles (movement restriction of the bulbi, double images). Endocrine orbitopathy is often combined with pretibial myxedema, which pathogenetically is based on an identical mechanism.

Clinical features
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Eye strain, burning eyes, photophobia, increased lacrimation, lid edema, double vision. Other signs as a consequence of exopthalmia: less frequent blinking (Stellwag sign), receding of the upper eyelid when lowering the gaze (Graefe sign), weakness of convergence (Möbius sign).

Furthermore, in >90% of cases the signs of immunogenic hyperthyroidism (Graves' sign) are detected.

Pretibial myxoedema is rarer (although rare, it can be located on the forearms or shoulders).

Very rare is acropachia (club-shaped distension of the finger/toe phalanges (Elsayad K et al. 2015) .

6 degrees of severity are distinguished:

  1. Anamnestic complaints like foreign body sensation, increased tearing, retrobulbar pressure sensation, light sensitivity
  2. Conjunctivitis, chemosis (= edema of the conjunctiva), conjunctivitis, periorbital swelling, thickening of the lacrimal glands
  3. Protusio bulbi (light, clear, very distinct)
  4. Eye muscle blockades with double images
  5. Corneal ulcerations with double images
  6. Loss of visual acuity with blindness

Laboratory
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TRAK (TSH receptor antibody) positive; the higher the TRAK level, the more active the EO.

Often hyperthyroidism, possibly also euthyroidism, rarely hypothyroidism.

Therapy
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Field of ophthalmology. No causal therapy known.

Establish euthyroid metabolism (thyrostatic drugs).

Immunosuppressive therapy is partially successful.

Possibly retrobulbar irradiation of the orbit with omission of the eye lens

Progression/forecast
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Uncertain. 30% improvement, 60% no change, 10% deterioration.

Note(s)
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There are associations with the EMO syndrome, whereby myxoedema or acropacity can occur in varying degrees.

There is an 8-fold increased risk of EO in patients with immunogenic hyperthyroidism (M. Basedow) and smoking.

Literature
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  1. Elsayad K et al (2015) Radiation therapy as part of the therapeutic regimen for extensive multilocular myxedema in a patient with exophthalmos, myxedema and osteoarthropathy syndrome: A case report. Oncol Lett 9: 2404-2408.
  2. Guerrero C et al (2016) IMAGES IN CLINICAL MEDICINE. Thyroid ophthalmopathy, dermatopathy, and acropachy. N Engl J Med 375(3):261.
  3. Liu X et al (2016) Clinical efficacy of combined rituximab treatment in a woman with severe Graves' ophthalmopathy. Exp Ther Med 12:1093-1096.
  4. Sabini E et al (2016) Statins are not a risk factor for liver damage associated with intravenous glucocorticoid pulse therapy for Graves' orbitopathy. J Endocrinol Invest PubMed PMID: 27465669.
  5. Tamagno G et al(2004) Graves' ophthalmopathy andatrophic thyroiditis: a case report. J Endocrinol Invest 27:163-166.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020