Images (2)
Graves' ophthalmopathyE05.0 H06.2
Synonym(s)
DefinitionThis section has been translated automatically.
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).
EtiopathogenesisThis section has been translated automatically.
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 featuresThis section has been translated automatically.
Eye pressure, burning eyes, photophobia, increased lacrimation, eyelid edema, double vision. Further signs as a consequence of exopthalmos: infrequent blinking (Stellwag's sign), receding of the upper eyelid during lowering of the gaze (Graefe's sign), weakness of convergence (Möbius' sign).
Furthermore, signs of immunogenic hyperthyroidism (Graves' disease) are detected in >90% of cases.
Less common is pretibial myxedema (this may be localized, although rarely, on forearms or shoulders).
Very rare is acropachy (club-shaped distension of the fingers/toe ephalanges (Elsayad K et al. 2015) .
6 degrees of severity are distinguished:
- Anamnestic complaints such as foreign body sensation, increased tearing, retrobulbar pressure sensation, photosensitivity
- Conjunctivitis, chemosis (= edema of the conjunctiva), conjunctivitis, periorbital swelling, thickening of the lacrimal glands
- Protusio bulbi (mild, marked, very pronounced)
- ocular muscle blockages with double images
- corneal ulceration with double vision
- Loss of visual acuity with blindness
LaboratoryThis section has been translated automatically.
TRAK (TSH receptor antibody) positive; the higher the TRAK level, the more active the EO.
Often hyperthyroidism, possibly also euthyroidism, rarely hypothyroidism.
TherapyThis section has been translated automatically.
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/forecastThis section has been translated automatically.
Uncertain. 30% improvement, 60% no change, 10% deterioration.
Note(s)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- 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.
- Guerrero C et al (2016) IMAGES IN CLINICAL MEDICINE. Thyroid ophthalmopathy, dermatopathy, and acropachy. N Engl J Med 375(3):261.
- Liu X et al (2016) Clinical efficacy of combined rituximab treatment in a woman with severe Graves' ophthalmopathy. Exp Ther Med 12:1093-1096.
- 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.
- Tamagno G et al(2004) Graves' ophthalmopathy andatrophic thyroiditis: a case report. J Endocrinol Invest 27:163-166.