Thyroid Eye Disease

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What is TED?

Thyroid eye disease (TED) is the most common cause of orbital inflammation and proptosis in adults, accounting for approximately 40% of all orbital disease.  At least 80% of TED is associated with Graves’ disease (GD), one of the most common endocrine abnormalities in the world.  Fifty to ninety percent of GD patients develop clinically evident, symptomatic, TED. GD and TED affect over 20 million previously healthy and productive adults in the United States alone. The peak incidence is in the fourth and fifth decades but may affect any race and any age.

Stabilization typically occurs in the second or third year but may be delayed in:

- Men
- Older patients (> 50 years old)
- Cigarette smokers
- Those with prolonged hypothyroidism or poorly controlled thyroid functions

Typical symptoms

- Visual blurring and distortion
- Double vision
- Tearing
All of the above may interfere with driving, reading and computer use. 

Typical signs:

- Bulging eyes (proptosis)
- Staring appearance (eyelid retraction causing more of the white of the eyes (sclera) to show)
- Misaligned eyes from enlarged, restricted muscles behind the eyeball

TED can alter lives

- Can result in visual disability
- Can disfigure patients
- Many TED patients are depressed, withdraw socially, lose self-esteem and suffer job dislocation, divorce and emotional devastation
-The economic effect of this disease is great because it affects women, and less commonly men, in their peak years of productivity, impacting their families, communities and workplaces.

Management of TED

The most common medical interventions - corticosteroids and/or external beam radiation - are typically reserved for patients with a vision threatening active phase or rapidly progressive orbitopathy. 
Rationale for Using Corticosteroids for TED.

-Oral corticosteroid treatment (usually Prednisone) has been associated with a short term decrease in soft tissue swelling, optic neuropathy and extraocular muscle size in up to 65% of cases
- The clinical response to intravenous corticosteroids is as high as 85% with fewer side effects than oral Prednisone

Rationale for Using External Beam Radiation.

Data addressing the efficacy of orbital irradiation, either alone or in combination with other treatment modalities (corticosteroids, steroid sparing agents, surgery) is conflicting. Overall improvement rates of 60-65% are typical in reported studies treating TED patients in the active phase of the disease. Radiation can decrease redness, swelling and discomfort; but is not routinely effective in reducing proptosis or double vision. Radiation can also be used effectively if optic neuropathy is present in an actively inflamed TED patient. Radiation is contraindicated in Diabetics.

The Role of Surgery in Thyroid Eye Disease

In most cases, surgery is delayed and is targeted at modification of stable phase dysfunction.
Orbital Decompression surgery is effective in decreasing or eliminating the bulging (proptotic) eyes.

Eyelid retraction repair surgery is effective in decreasing or eliminating the stare that is so characteristic of TED. The eyelids are often staying open at night and causing break down of the surface of the eye.

Eye muscle surgery is performed to restore single vision when looking straight ahead and down. Unfortunately, there is no way to return the muscle function to normal – so double vision in extremes of gaze is a common permanent change.

Although late surgical interventions can help decrease disfigurement and allow the patient to see a single image when looking straight ahead, the tissue alterations are irreversible. There is no way to restore normal eye muscle movement or pre-disease appearance.  Thus, many of the manifestations of TED are permanent.

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Why did I develop TED?

Simple reason: TED is an autoimmune disease where your body is treating the tissues around the eye as foreign and attacking them.

The complex science as we know it today:

Graves’ disease is a systemic autoimmune disease associated with dysregulation and over activity of the thyroid gland, and extensive remodeling of orbital and pretibial dermal connective tissues. The pathogenic relationship between the thyroid dysfunction and inflammation of the orbital tissues is an enigma.  The immune response in TED is both humoral and cell-mediated. Monocytes, lymphocytes and mast cells are known to participate but the mechanism for the activation and trafficking of these cells is unknown.

The acute phase clinical features of TED result from mononuclear cell (primarily lymphocytes) infiltration of the orbital fat, extraocular muscles and periorbital tissues. The site specific response, in the orbit and pretibial dermis, to the systemic disease is explained in part by the unique phenotype of the resident fibroblasts which in the setting of TED respond to pro-inflammatory cytokines at a 100-fold greater rate than abdominal fibroblasts. In TED, orbital fibroblasts orchestrate the recruitment of immuno-competent cells and initiate tissue remodeling.  Activated fibroblasts drive the disease process through expression of molecular mediators including cytokines and chemoattractants, that both promote ongoing inflammation and immune recruitment.

Orbital fibroblasts, in TED, promote the accumulation of the glycosaminoglycan, hyaluronan, in the orbital fat and extraocular muscles in response to proinflammatory mediators.  Inflammation-mediated production and accumulation of orbital glycosaminoglycans is the hallmark of active TED. The highly charged, hydrophilic, hyaluronan molecules attract water, and thereby contribute to orbital tissue expansion and orbital congestion. Orbital soft tissues are further expanded through the enlargement of the intraconal fat compartment by proliferation of pleuripotent preadipocyte fibroblasts acting under the same inflammatory stimulation. Soft tissue expansion results in proptosis, restricted eye muscle movement and, in some patients, optic neuropathy.  Irreversible changes in orbital dynamics occur through the third mechanism of fibroblast activity, fibrosis, occurring at the level of the fibrovascular matrix of the orbital soft-tissue. Consequently, the inflammatory events mediating orbital fibroblast activation are an attractive therapeutic target.

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What is TSI and Why is it tested?

Laboratory tests to detect thyroid dysfunction, including thyroid stimulating hormone, thyrotropin, and serum free T3 and T4 levels, do not correlate with TED disease activity.

Thyroid stimulating immunoglobulins (TSI), in contrast, have been found in more than 90% of patients with active GD and in 50-90% of euthyroid TED patients.

Also, TSI levels are significantly higher in Graves (GD) patients with TED; higher TSI levels also correlate with the presence of active TED signs such as eyelid edema, eye muscle enlargement and proptosis.

Patients with active TED often have a TSI values three to five times that of normals (reference value is 125). TSI levels drop as TED becomes inactive.

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