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Sympathetic ophthalmia (SO), also called spared eye injury, is a diffuse
granuloma A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectious o ...
tous inflammation of the uveal layer of both eyes following trauma to one eye. It can leave the affected person completely blind. Symptoms may develop from days to several years after a penetrating eye injury. It typically results from a
delayed hypersensitivity Type IV hypersensitivity, often called delayed-type hypersensitivity, is a type of hypersensitivity reaction that can take a day or more to develop. Unlike the other types, it is not humoral (not antibody-mediated) but rather is a type of cell-me ...
reaction.


Signs and symptoms

Eye floaters and loss of accommodation are among the earliest symptoms. The disease may progress to severe inflammation of the uveal layer of the eye (uveitis) with pain and sensitivity of the eyes to light. The affected eye often remains relatively painless while the inflammatory disease spreads through the uvea, where characteristic focal infiltrates in the choroid named Dalén–Fuchs nodules can be seen. The retina, however, usually remains uninvolved, although perivascular cuffing of the retinal vessels with inflammatory cells may occur. Swelling of the optic disc (papilledema),
secondary glaucoma Secondary glaucoma is a collection of progressive optic nerve disorders associated with a rise in intraocular pressure (IOP) which results in the loss of vision. In clinical settings, it is defined as the occurrence of IOP above 21 mmHg requiring t ...
,
vitiligo Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes. Signs and symptoms The only sign of vitiligo is the presence of pale patchy areas of depigmen ...
, and poliosis of the eyelashes may accompany SO.


Pathophysiology

Sympathetic ophthalmia is currently thought to be an
autoimmune In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". ...
inflammatory response toward ocular
antigens In immunology, an antigen (Ag) is a molecule or molecular structure or any foreign particulate matter or a pollen grain that can bind to a specific antibody or T-cell receptor. The presence of antigens in the body may trigger an immune response. ...
, specifically a delayed
hypersensitivity Hypersensitivity (also called hypersensitivity reaction or intolerance) refers to undesirable reactions produced by the normal immune system, including allergies and autoimmunity. They are usually referred to as an over-reaction of the immune ...
to
melanin Melanin (; from el, μέλας, melas, black, dark) is a broad term for a group of natural pigments found in most organisms. Eumelanin is produced through a multistage chemical process known as melanogenesis, where the oxidation of the amino ...
-containing structures from the outer segments of the photoreceptor layer of the
retina The retina (from la, rete "net") is the innermost, light-sensitive layer of tissue of the eye of most vertebrates and some molluscs. The optics of the eye create a focused two-dimensional image of the visual world on the retina, which the ...
. The immune system, which normally is not exposed to ocular proteins, is introduced to the contents of the eye following traumatic injury.A cautionary tale from the past
by
K. Barry Sharpless Karl Barry Sharpless (born April 28, 1941) is an American chemist and a two-time Nobel laureate in Chemistry known for his work on stereoselective reactions and click chemistry. Sharpless was awarded half of the 2001 Nobel Prize in Chemistry ...
, in
MIT Tech Talk MIT Tech Talk (abbreviated Tech Talk) was the Massachusetts Institute of Technology's official newspaper from February 1957 until September 2009, when it ceased publication. It was written and published by the MIT News Office, both in print and on ...
; published March 19, 1992; retrieved May 10, 2016
Once exposed, it senses these antigens as foreign, and begins attacking them. The onset of this process can be from days to years after the inciting traumatic event.


Diagnosis

Diagnosis is clinical, seeking a history of eye injury. An important differential diagnosis is Vogt–Koyanagi–Harada syndrome (VKH), which is thought to have the same pathogenesis, without a history of surgery or penetrating eye injury. Still experimental, skin tests with soluble extracts of human or bovine uveal tissue are said to elicit delayed hypersensitivity responses in these patients. Additionally, circulating antibodies to uveal antigens have been found in patients with SO and VKH, as well as those with long-standing uveitis, making this a less than specific assay for SO and VKH.


Treatment

Because SO is so rarely encountered following eye injury, even when the injured eye is retained, the first choice of treatment may not be enucleation or evisceration, especially if there is a chance that the injured eye may regain some function. Additionally, with current advanced surgical techniques, many eyes once considered nonviable now have a fair prognosis. However, only if the injured eye has completely lost its vision and has no potential for any visual recovery, prevention of SO is done by enucleation of the injured eye preferably within the first 2 weeks of injury.
Evisceration Evisceration (pronunciation: /ɪvɪsəˈreɪʃən/) is disembowelment, i.e., the removal of viscera (internal organs, especially those in the abdominal cavity). The term may also refer to: * Evisceration (autotomy), ejection of viscera as a defe ...
—the removal of the contents of the globe while leaving the sclera and
extraocular muscles The extraocular muscles (extrinsic ocular muscles), are the seven extrinsic muscles of the human eye. Six of the extraocular muscles, the four recti muscles, and the superior and inferior oblique muscles, control movement of the eye and the oth ...
intact—is easier to perform, offers long-term orbital stability, and is more aesthetically pleasing, i.e., a greater measure of movement of the prosthesis and thus a more natural appearance. There is concern, however, that evisceration may lead to a higher incidence of SO compared to enucleation. Several retrospective studies involving over 3,000 eviscerations, however, have failed to identify a single case of SO. Once SO is developed, immunosuppressive therapy is the mainstay of treatment. When initiated promptly following injury, it is effective in controlling the inflammation and improving the prognosis. Mild cases may be treated with local application of
corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...
and pupillary dilators. More severe or progressive cases require high-dose systemic corticosteroids for months to years. Patients who become resistant to corticosteroids or develop side effects of long-term corticosteroid therapy (osteoporosis and pathologic fractures, mental status changes, etc.), may be candidates for therapy with
chlorambucil Chlorambucil, sold under the brand name Leukeran among others, is a chemotherapy medication used to treat chronic lymphocytic leukemia (CLL), Hodgkin lymphoma, and non-Hodgkin lymphoma. For CLL it is a preferred treatment. It is given by mouth. ...
,
cyclophosphamide Cyclophosphamide (CP), also known as cytophosphane among other names, is a medication used as chemotherapy and to suppress the immune system. As chemotherapy it is used to treat lymphoma, multiple myeloma, leukemia, ovarian cancer, breast cancer ...
, or
ciclosporin Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is a natural product. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease ...
.


Epidemiology

Sympathetic ophthalmia is rare, affecting 0.2% to 0.5% of non-surgical eye wounds, and less than 0.01% of surgical penetrating eye wounds. There are no gender or racial differences in incidence of SO.


History

Although descriptions of sympathetic ophthalmia can be found in ancient Greek texts, modern understanding of SO derives from the works of Scotland's William MacKenzie who characterized and named the disease sympathetic ophthalmitis. At MacKenzie's time, oral mercury and
leeches Leeches are segmented parasitic or predatory worms that comprise the subclass Hirudinea within the phylum Annelida. They are closely related to the oligochaetes, which include the earthworm, and like them have soft, muscular segmented b ...
applied to the
conjunctiva The conjunctiva is a thin mucous membrane that lines the inside of the eyelids and covers the sclera (the white of the eye). It is composed of non-keratinized, stratified squamous epithelium with goblet cells, stratified columnar epithel ...
were the treatments of choice for SO. It is thought that
Louis Braille Louis Braille (; ; 4 January 1809 – 6 January 1852) was a French educator and the inventor of a reading and writing system, named braille after him, intended for use by visually impaired people. His system is used worldwide and remains virtu ...
, who injured one of his eyes as a child, lost vision in his other eye owing to SO.
James Thurber James Grover Thurber (December 8, 1894 – November 2, 1961) was an American cartoonist, writer, humorist, journalist and playwright. He was best known for his cartoons and short stories, published mainly in ''The New Yorker'' and collected ...
's adult blindness was also diagnosed as sympathetic ophthalmia deriving from the loss of an eye when he was six years old.


See also

* Vogt–Koyanagi–Harada disease


References


External links

{{DEFAULTSORT:Sympathetic Ophthalmia Eye diseases Autoimmune diseases