Ocular sarcoidosis

Introduction to ocular sarcoma Sarcoidosis, also known as sarcoidosis, was first proposed by PMBoeck in 1899. In 1914 Schaumann classified benign lymphogranulomatosis damaged by the reticuloendothelial system into sarcoma. The name ocular sarcoma has been recognized. Ocular sarcoma disease can invade various organs such as human lung, lymph nodes, skin, nerve spleen, liver heart, parotid gland, muscle, bone and bone joints, and epithelial-like cells that do not undergo cheese-like transformation, also known as granuloma formed by epithelial cells. Characterized. The eye is the vulnerable organ that is second only to the lung and hilar lymph nodes. When the eye is sick, it is usually accompanied by sarcoma of other organs but in some cases, the eye disease is very typical and the whole body examination has no positive findings. Ocular sarcoma disease. basic knowledge The proportion of sickness: 0.00004% - 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: glaucoma cataract

Causes of ocular sarcoma

Causes:

The cause is still unclear. The current hypothesis is that sarcoma-like disease is caused by abnormal pathogenic immunity and is caused by a certain pathogen infection. Among various pathogens, the most controversial is tuberculosis, which has been considered sarcoma in history. The disease is a rare form of tuberculosis infection, but tuberculosis can not be isolated or rarely found in sarcoma-like granuloma; no or only weak response to tuberculin test; ineffective treatment of anti-tuberculosis drugs, etc. Bacteria are presumed to be the cause of the disease. In addition, the association between atypical mycobacteria and certain herpes viruses such as herpes simplex, Epstain-Barr virus infection and this disease has not been fully confirmed.

The disease has an immune genetic background. In 1997, Ishihara and other people surveyed in the Nordic region found that HLA-B8, HLA-DR positive, susceptible to sarcoma.

Pathogenesis:

Recent studies have shown that the disease is an autoimmune disease, and autoantigens may be associated with monocytes. The immunological changes in patients are characterized by a decrease in CD4 cells in peripheral blood and a decrease in the ratio of CD4 cells to CD8 cells (= 0.8), but the CD4+ cells increased in the affected tissues, and the ratio of CD4 cells to CD8+ cells increased (=1.8). Studies have shown that CD4 cells in the affected tissues are activated, releasing IL-2 and other cytokines, mainly caused by cellular immune responses. Inflammation and tissue damage.

The differences in the incidence of different races and the clinical manifestations of different sarcomatoid patients suggest that immune genetic factors play a role in the pathogenesis. HLA antigen typing found that HLA-B8 antigen-positive people are prone to acute in white patients in London. Arthritis, nodular erythema, and anterior uveitis; HLA-Bw15 antigen-positive people in Washington blacks are five times more likely to develop sarcoma than those who are negative; in Greek patients, HLA-B27 antigen-positive There are often more serious sarcoma-like diseases, and HLA-B13 antigen-positive patients often show chronic and persistent damage.

Ocular sarcoma prevention

Pay attention to the usual habits. Pay attention to maintain adequate sleep, avoid overwork, pay attention to work and rest, pay attention to the regularity of life.

Ocular sarcoma complications Complications glaucoma cataract

Secondary glaucoma is the most common complication, and complicated cataracts can also occur.

Symptoms of ocular sarcoma disease Common symptoms Eye pain, photophobia, tear, lymph node enlargement, uveitis, ciliary congestion, secondary optic atrophy

Anterior segment of uveal inflammation

(1) sarcoma-like iridocyclitis: iridocyclitis (hereinafter referred to as iriditis) is the most common ocular disease of sarcoma-like disease, accounting for 65.2% of sarcoma-like eye diseases. For young people, mostly single eyes, after a seizure, recurrence, ciliary congestion at the time of onset, pale white corneal depression, positive Tyndall phenomenon, unclear iris texture, dilated pupils, eye pain, tenderness in the ciliary region Inflammatory irritations and signs of iridescent inflammation such as photophobia, tearing, etc., the body is often accompanied by skin nodular erythema and hilar lymphadenopathy. Chronic erythematosus is different from acute ones: after the middle age, most of them Both eyes, at the same time or successively, the disease course persists, ciliary congestion, eye pain and other symptoms and signs of inflammatory irritation are not significant, KP is densely packed with sheep fat, iris Koeppo nodules and Busacca nodules are quite common, iris is easy to post Adhesion, therefore also prone to complicated cataract, secondary glaucoma, systemic examination can find pulmonary fibrosis lesions, lupus pernio (lupus pernio), bone cysts and other lesions.

(2) sarcoma-like trabecular inflammation: 4.9% to 8.5% of patients with sarcoma, granulomatous nodules appear at the Schwalbe line and trabecular meshwork below the iris corneal angle, and Candler calls it sarcoma-like trabeculae Inflammation, the nodules are slightly reddish yellow translucent miliary ridges, varying in length, sometimes merging, and nodules near the nodules can be seen in the vicinity of the nodules, and the iris corneal angles are mostly open angles. However, when the fibrotic anterior adhesion is severe, it can be closed. The intraocular pressure generally remains normal and can be increased. No matter whether the intraocular pressure is increased or not, there is no congestion in the anterior segment of the eye, and there is no eye pain.

(3) Ciliary body sarcoma nodules: granulomatous nodules have different findings due to whether they break through the pigment epithelial layer, and the breakthrough nodules are larger, otherwise small nodules, indirect ophthalmoscopes or three-sided mirrors are difficult to see. To the ciliary body, ultrasound biomicroscopy can be used.

(4) Uveitis of the sarcoma-like disease: in terms of signs and symptoms, it is basically consistent with the common idiopathic uveal inflammatory disease. The difference lies in the small snowball-like exudation group of the disease, located at the base of the vitreous. Close to the retina, about 1/5PD in size, often in a beaded arrangement, unlike the snow bank-like exudation of idiopathic uvitis in the flat portion of the ciliary body.

2. Inflammation of the posterior uveal ganglion caused by inflammatory sarcoma in the posterior uveal ganglia is less common than in the anterior segment. The incidence of anterior and posterior inflammatory disease is about 4:1. There is often no or only mild anterior segment in the posterior segment of inflammation. Inflammation.

(1) sarcoma-like chorioretinitis: a round or round yellow-white nodule with clearer boundaries at the endoscope, with an area of 1/3 to 1/2 PD, like a wax droplet, called a wax tear (candle wax driping), the wax teardrop is located near the retinal vein, which varies somewhat, and can be absorbed by itself within a few months. It can also be changed over several years. This lesion may be a granuloma of the vascular wall epithelioid cells, on the FFA. The existing blood vessel wall defects, after the disappearance of wax tears, left yellow-white atrophy, few pigment hyperplasia, scattered in the vitreous, spherical gray-white exudate ranging from small dots to 1PD, often connected Beaded, mostly located in front of the retina below the fundus, the projection can be seen when the ophthalmoscope is examined. This is one of the characteristics of the disease. In some cases, there are small nodules at the trabecular meshwork of the iris cornea. This is another feature of the disease, so the iris Keratoscopic examination is useful for the diagnosis of this disease.

(2) sarcoma-like retinal vein inflammation: In the fundus lesions caused by sarcoma-like disease, the most common retinal vein inflammation is accompanied by white sheath and hemorrhage along the vein, and the peripheral venules are occluded into white lines. Wax teardrops and bead-like beads exuded in the vitreous. In some cases, it was also found that when the inflammation damaged the larger vein branches, there was occasional vitreous hemorrhage.

Sarcomatoid disease rarely involves the retinal artery. When the venous wall is seen as a segmental white sheath, it is called sarcoma retinal vasculitis.

(3) sarcoma-like choroidal granuloma: There are two types: one is scattered multiple choroidal nodules, the position is deep, dark yellow, ICG angiography is low in the early stage, strong fluorescence in the late stage; The nodules are large, up to 8PD, yellow-white or gray-yellow, the retina can be detached, and the retina can be broken into the vitreous, which is easily misdiagnosed as a choroidal malignancy.

Choroidal granuloma is not associated with anterior uveal inflammation. FFA examination begins with fluorescence obscuration, followed by leakage (new blood vessels on the surface of granuloma), late infection, and large choroidal retinal atrophy and pigmentation after granulomatosis subsides.

(4) Optic nerve damage of sarcoma-like disease: most of them are optic disc edema or optic neuritis secondary to sarcoma-like chorioretinitis; also due to intracranial and sputum sarcoma, causing intracranial or intra-orbital pressure to increase Or monocular optic disc edema, if long-term can not be alleviated, can eventually develop secondary optic atrophy, in some cases the optic nerve directly affected by sarcoma-like disease, the formation of optic nerve granuloma, due to optic nerve fibers are squeezed, not only damage visual function, but also Involving the pupillary reflex in the pupil, the granuloma in the anterior segment of the optic nerve, the optic disc under the ophthalmoscope, slightly pink, severely protruding into the vitreous cavity, sometimes due to granuloma compression, can also cause retinal vein occlusion, sarcoma-like disease Posterior optic neuritis may also be caused by small granuloma in the posterior optic nerve. The inner part of the optic canal and the granuloma of the intracranial segment. In addition to the sharp decrease in visual acuity, changes in the optic canal can be seen on the CT or MRI. , there is a corresponding change in visual field, accompanied by hypothalamic, pituitary, endocrine symptoms and signs.

Examination of ocular sarcoma

1. Biochemical examination

(1) Serum angiotensin-converting enzyme: serum angiotensin-converting enzyme (ACE) is produced by macrophages, epithelial cells, etc., and can catalyze the cleavage of various polypeptides, which can convert angiotensin I into Angiotensin II, a potent vasopressor, is normally present on the surface of pulmonary capillary endothelial cells, alveolar macrophages, epithelial cells of proximal tubules, blood and semen, in patients with sarcoma, due to A large number of granuloma containing macrophages and epithelial cells and the hyperfunction of these cells, so that not only a high concentration of ACE in the granuloma tissue, but also a significant increase in serum ACE levels, in a sense, Serum ACE levels reflect the sum of granuloma (epithelial cells, macrophages) that produce ACE. Since Lieberman introduced the ACE assay into the diagnosis of sarcoma in 1974, this type of examination has received widespread attention and it has become A useful indicator for judging sarcoma-like disease, dynamic determination of serum levels, can determine the progress of the disease, regression and recurrence, can evaluate the rind Hormone treatment can be used as a prognostic parameter determination can also be used as an aspect of the mechanism of category sarcoma Disease.

(2) Lysozyme: The level of lysozyme in serum and other body fluids reflects the activity of macrophages in the body. In patients with sarcoma-like diseases, the number of these cells increases and the activity is enhanced, so that serum lysozyme levels may increase.

Lysozyme assay has been applied to the differential diagnosis of uveitis and keratoconjunctivitis. Both sarcoma-like uveitis and elevated serum lysozyme levels should be highly suspected to be sarcoma-like; The lysozyme content of tears in patients with keratoconjunctivitis caused by sarcoma is normal or elevated, but the levels of tear lysozyme in patients with dry keratoconjunctivitis caused by other types are reduced.

(3) adenosine deaminase: adenosine deaminase (ADA) is mainly produced by macrophages and T lymphocytes, Tayler et al found that serum ADA activity increased in patients with sarcoma.

2. Immunological examination

(1) humoral immunological examination: patients with sarcomatoid disease have abnormal humoral immunity. Because auxiliary T cells stimulate a variety of B cells, the levels of various types of immunoglobulin in serum are increased, and many patients have rheumatoid factor. , anti-nuclear antibodies, syphilis antibodies and other false positive results and a variety of viral antibody titers, in some patients with serum and aqueous humor immune complexes, but because of the sensitivity of these tests vary greatly, therefore, in sarcoma The value in diagnosis is not great.

(2) Cellular immunological examination: about 1/3 of patients have mild lymphopenia, and lymphocytes respond to non-specific mitogen stimulation, but because of the lack of specificity of these tests, it does not help the diagnosis. Skin tests have been helpful in this disease. Most active patients have no response to intradermal tests such as Candida, mumps virus, and Trichophyton; about 90% of normal people have positive for streptokinase intradermal test. Only half of the patients were positive; with the 100 U tuberculin skin test, about 2/3 of the patients were negative, and the positive patients were negative as the disease worsened, but after the disease activity disappeared, Can be converted to positive.

(3) Kveim test: The Kveim test is actually a test for delayed skin allergic reaction. It was first described in 1941. This test overcomes the shortcomings of other skin tests with low sensitivity and specificity. It is reported that 50%~ 80% of patients are positive and up to 90% positive in patients with pulmonary sarcoma. Therefore, it is of great value in the diagnosis of sarcoma-like disease. This test is based on the pathological tissue of patients with sarcoma-like disease (usually using spleen). And lymph nodes) extract protein or prepare tissue suspension, inject protein or tissue suspension into the skin of the patient, observe the results after 4 to 6 weeks, and the local appearance of malignant nodules is positive, and the nodule biopsy can be seen. In the case of non-caseous necrotizing granuloma, in the early stage of the presence of lymph node lesions and nodular erythema, this test is mostly positive. The false positive rate of this test is low, and the false positive is only found in patients with infectious intestinal diseases, tuberculosis, etc. Although the test is very helpful for diagnosis, it has the following shortcomings and limitations:

1 It is difficult to obtain the spleen and lymph nodes of patients with sarcoma-like disease, and even if it is necessary to prove the usefulness of this antigen in a large number of patients, there is currently no spleen or lymph node suspension or antigen of a commercially available patient;

2 It takes at least 1 month to observe the results;

3 requires a histological examination, Jones-Williams et al designed an in vitro test method called Kveim macrophage inhibitory factor test, it is said that it can get results faster than the standard Kveim test, can avoid live Tissue examination and histological examination to avoid the transmission of pathogens such as hepatitis virus, but more research and confirmation are needed to confirm the accuracy and validity of this test.

3. The blood erythrocyte sedimentation rate is accelerated, the total number of white blood cells is decreased, eosinophils are increased, the ratio of serum globulin to albumin is inverted, and and globulin are elevated, which can provide reference for diagnosis.

4. Histopathological examination occurs in any organ of sarcoma-like granulomatous nodules, histological changes are consistent, formed by the reaction between antigen and macrophages, T cells, and antibodies.

Under the microscope, the granuloma nodules are mainly composed of skin-like cells. Some of them have multinucleated giant cells, and there are lymphocytes, monocytes and fibroblasts infiltrated in the periphery. Small nodules can be aggregated into large spheroid nodules. In the nodules of the specimen, the giant cells have birefringent crystalline inclusion bodies, namely Schaumann bodies, and the sarcoma-like granulomatous nodules do not undergo cheese-like changes but have Schaumann bodies, which are distinguished from the histopathological features of tuberculous nodules. The main basis, but not absolute, is also seen in tuberculosis nodules with low toxicity, high immunity and low tissue allergies; also found in fungal infections, leprosy, toxoplasmosis, In the granulomatous nodules formed by sporozoites, beryllosis and foreign bodies, granular necrosis sometimes occurs in nodules of sarcoma.

The granulomatous nodular granulomatous nodules that occur in the iris, ciliary body, choroid, retinal neuroepithelial and pigmented epithelium (RPE), as seen in the histopathology of other organs, clinically known as scattered multiple choroid nodules The lesion is confined to the retinal pigment epithelial cells and does not actually invade the choroid. In the retinal vein inflammation or vasculitis specimens, nodular granulomatous infiltration can be seen in the wall, and even the vessel wall is completely replaced by epithelial cells. However, there are also individual cases in which the entire retina is interspersed with miliary nodules without affecting blood vessels.

Sarcoma-like acute iridoxitis is non-granulomatous inflammation, and histopathological changes are like skin nodular erythema, which is a secondary response to immune sensitization.

Immunohistochemistry suggests that epithelioid cells in the sarcoma granulomatous nodules of sarcoma-like sarcoma have T lymphocytes derived from macrophages, and the number of helper T lymphocytes is significantly higher than that of inhibitory T lymphocytes. Around the granuloma nodules.

X-ray inspection

(1) Chest X-ray examination: It is one of the most important examination methods. According to the X-ray performance, the lung lesions caused by sarcoma-like disease can be divided into the following five stages: stage 0, no X-ray abnormality; stage I Only bilateral hilar lymphadenopathy occurred, no lung field changes, this lesion was 80% to 90% self-healing in 1 year; Phase II, with both hilar lymphadenopathy and lung field changes, lung field changes Polymorphism, but interstitial plaque or nodular infiltration is more common, lung field lesions are not specific, such as lung field lesions prior to bilateral hilar lymphadenopathy, should be considered tuberculosis, fungi Infection, lymphoma, cancer, etc.; stage III, only lung field lesions without hilar lymphadenopathy; stage IV, lung field showed the above changes and fibrosis, although stage IV can be developed from stage I, II However, in fact, this stage is only artificial division, does not indicate the development order of the lung lesions of this disease, stage 0 can occur in the early or late stage of the disease, in patients with sarcomatoid disease, oligo-tracheal lymphadenopathy rarely occurs No hilar lymphadenopathy, in addition, hilar lymphadenopathy always appears in the lung field Before, this is the key difference between the disease and other diseases caused by lung disease.

(2) X-ray examination of bone: Some patients may have bone involvement occasionally, and the main three types of X-ray findings are:

1 reconstruction of the cortical funnel and trabecular bone;

2 dissolution phenomenon, manifested as a perforated edge;

3 quickly destroy the phenomenon.

2. Gallium scan of gallium citrate scan has certain value for the discovery of systemic inflammatory diseases and tumors. Although the exact mechanism of gallium localization is not fully understood, it is known that it may be related to T cells in the inflammation site, and the amount of gallium absorption depends. In the characteristics and extent of inflammation, gallium is particularly suitable for the detection of inflammation caused by sarcoma-like disease. It has been found to be more sensitive than X-ray in the diagnosis of lung lesions caused by sarcoma-like disease, but lymphoma, cancer, tuberculosis and other diseases. The gallium scan can also change abnormally.

For patients with suspected sarcoma, a lacrimal gland scan should be performed to determine whether there is a change in the lacrimal gland. A small amount of gallium absorption can be seen in the normal lacrimal gland tissue. In patients with active sarcoma, more than 4/5 of the gallbladder absorption increases abnormally, indicating The involvement of lacrimal gland in patients with sarcoma is quite common. Although the lacrimal gland scan is a sensitive method, it is non-specific, about 25% of lacrimal gland absorption increases, no sarcoma-like manifestations, so it should be noted This false positive result.

3. CT and MRI For granuloma of the intracranial and optic nerves, CT or MRI can be performed to help identify granulomas and tumors.

Diagnosis and diagnosis of ocular sarcoma

diagnosis

Although there are some characteristic changes in the sarcomatoid inflammation of the anterior and posterior segments of the uveal, it is difficult to see uveitis, chorioretinitis, retinal vasculitis, and other causes, just by slit lamp microscopy and ophthalmoscopy. Optic neuropathy, therefore, must be combined with systemic examination, when uveitis with skin nodular erythema, plaque papules, chest X-ray or CT, found bilateral hilar lymphadenopathy, must be highly suspected uveitis may be a class Caused by sarcoma, if the tuberculin test is negative, Kveim test is positive, the diagnosis can be established.

Differential diagnosis

1. syphilitic uveitis syphilis can cause serous iridocyclitis, granulomatous iridocyclitis, retinal choroiditis, retinal vasculitis, etc., can also cause some systemic changes in the identification of syphilis and sarcoma In the case of uveitis caused by disease, in addition to the history of attentional exposure and the characteristics of uveitis itself, the following aspects should be noted:

1 syphilis caused by lung lesions is very rare, and the lung changes caused by it are mainly bronchial syphilis, and the lung changes caused by sarcoma-like disease are mainly hilar lymphadenopathy;

2 syphilis patients with conjunctival or lymph node biopsy showed a large number of plasma cell infiltration, while patients with sarcoma were non-caseous necrotizing granuloma;

3 Conjunctivitis caused by syphilis usually manifests as severe congestion, and sarcoma-like disease is mainly yellow translucent nodules;

4 penicillin drugs are effective for the treatment of syphilitic uveitis, but not for sarcoma-like diseases;

5 syphilis serology and serum ACE tests are helpful for identification.

2.Vogt-Koyanagi Harada disease Vogt-Koyanagi Harada disease often causes bilateral granulomatous whole uveitis, diffuse choroiditis, may be accompanied by exudative retinal detachment, and the elderly with long-term changes in the course of the disease, Dalen The incidence of Fuchs nodules is much higher than that of patients with sarcoma-like disease. Patients often have headaches, tinnitus, hearing loss, hair loss, whitening of hair, vitiligo, and increased cerebrospinal fluid cells. According to these clinical features, Generally not difficult to identify.

3. Behcet's disease Umbilical disease Bechcet's disease is characterized by bilateral recurrent non-granulomatous anterior uveitis, total uveitis, retinitis, retinal vasculitis, recurrent oral ulcers, pleural skin lesions, Genital ulcers, arthritis, lymphadenopathy, changes in the central nervous system, skin allergic reaction test can be positive, no hilar lymphadenopathy, lung infiltration and other diseases, no serum ACE, lysozyme levels Elevated, fluorescein fundus angiography shows extensive retinal microvascular leakage, which contributes to the identification of both.

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