Subretinal fibrosis and uveitis syndrome

Introduction to subretinal fibrosis and uveitis syndrome Subretinal fibrosis and uveitis syndrome (subretinal fibrosis and uveitis syndrome) is a relatively rare clinical type characterized by the development of chronic uveitis and progression to subretinal fibrotic lesions. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: retinal detachment

Subretinal fibrosis and the etiology of uveitis syndrome

(1) Causes of the disease

still not clear.

(two) pathogenesis

Although the pathogenesis of this disease is not fully understood, some growth factors such as epidermal growth factor and -transforming growth factor caused by immune response and inflammation may be related to this type of fibrosis. Some people think that type III Allergic reactions play an important role in the development of antibodies to the retinal pigment epithelium or choroid-related components, which can form immune complexes and deposit in the retinal pigment epithelium and subretinal tissues, causing inflammatory reactions by activating the release of active products from complement. Tissue damage stimulates retinal vascular endothelium, glial cells, microglia, retinal pigment epithelial cells to proliferate, and forms subretinal fibrosis. Histological examination revealed B cell and plasma cell infiltration, supporting this view.

Another view is that delayed allergic reactions play an important role in their development; recently found in the subretinal fibrosis and uveitis patients' eye specimens, the retina, chorioretinal scar and choroidal granuloma Fas, FasL expression is increased, and it is believed that the interaction between the two may lead to gliosis or fibrosis.

Subretinal fibrosis and uveitis syndrome prevention

1, the close relatives of the marriage of the latent inheritance of the disease, its ancestors have a history of close relatives and smoke, prohibiting close relatives of cigarettes can reduce the incidence of this disease.

2, free of "sickness and disease combination." Patients with recessive inheritance should try to avoid marriage with family members of this disease, and can not marry those who also suffer from this disease. The risk of developing this disease in children with dominant hereditary diseases is.

3, blind patients should be treated early, because patients with retinitis pigmentosa manifest as night blindness in the early stage, often in children and adolescents, with increasing symptoms with age.

4, eat vitamin A, because vitamin A can prevent night blindness.

Subretinal fibrosis and complications of uveitis syndrome Complications

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Subretinal fibrosis and uveitis syndrome symptoms Common symptoms Fundus changes retinal edema Monocular anterior eye shadow unilateral fundus appears... Retinal detachment Uveitis visual distortion

Symptom

Most patients suffer from binocular involvement, but often complain of sudden or progressive unilateral vision blur or decline. The degree of visual acuity is very different. Some have only mild visual loss, while others can decrease to the immediate index or light perception. The patient may complain of a dark spot or multiple dark spots; there may also be visual distortion and a sense of flash.

2. Signs

At different stages of the disease, the fundus changes are very different. In the early stage, multiple small, scattered yellow-white lesions with blurred borders are located at the level of the retinal pigment epithelium and inner choroid, ranging from 50 to 500 m. They may also be arranged in clusters or in a line. The retinal pigment epithelial disorder has a mottled appearance. The lesions are mainly located in the posterior pole and the middle part. As the disease progresses, the yellow-white lesions can show several different types. Outcome: The first type does not leave any retinal pigment epithelial damage after the lesion has subsided (indicating that the lesion is in the choroid under the retinal pigment epithelium); the second is atrophic lesion with a perforated margin left after the lesion heals (indicating retinal pigment epithelial involvement) ), or the formation of chorioretinal scar; the third is the expansion of the lesion into an irregular band or large subretinal glial membrane, the lesion can affect the entire posterior pole, but also involving the middle part, activity The diseased fibrous membrane appears thick and dense and smooth, the border is slightly blurred, and the lesion is retinal edema, which may be accompanied by mild expansion of the retinal vessels. When it becomes static, the fiber membrane becomes thinner, appears dry and shrinking, and the boundary is clear, and the corresponding part of the retinal edema disappears; in some patients, the subretinal lesion can be arched around the macular area, which is different from the subretinal neovascular membrane. It rarely causes bleeding, but may be accompanied by macular area exudation and cystic edema, serous retinal detachment, optic disc edema, retinal vascular sheath may appear in some patients, after effective treatment, fibrosis under the retina tends to be static, Leaves a permanent fibrous film; the fourth is the subretinal neovascular membrane, which is prone to cause changes in bleeding, etc. In fact, the third case is the true subretinal fibrosis and uveitis syndrome.

There is usually no change in the anterior segment of the eye, but mild to moderate anterior uveitis may occur in a small number of patients, such as dusty KP, mild to moderate anterior chamber flash, a small amount of anterior chamber inflammatory cells and scattered iris After adhesion, some patients may have mild to severe vitreous inflammatory reactions, and the retina or retinal vessels may also be severely affected during the disease.

Examination of subretinal fibrosis and uveitis syndrome

1. Fluorescein fundus angiography in the acute phase of angiography, early display of high fluorescence and multifocal fluorescence masking, in some patients may appear plaque-like strong fluorescence, late lesions staining, with or without leakage There may be changes in optic fluorescein leakage, cystoid macular edema, etc.; old lesions often show staining.

2. Indocyanine green angiography currently has very little information on this aspect. Examination of 2 patients showed that the whole lesion showed weak fluorescence from early to late.

3. Electrophysiological examination of patients with visual acuity less than 0.2 or long-term need for glucocorticoid therapy, or non-response to glucocorticoids, often appear abnormal retinal current map and ocular electrogram.

4. Visual field examination Some patients may have dark spots, visual field defects, and some patients may have physiological blind spots to expand.

Diagnosis and differentiation of subretinal fibrosis and uveitis syndrome

Diagnostic criteria

Subretinal fibrosis and uveitis syndrome may be a common outcome of the development of many types of choroiditis, so attention should be paid to the diagnosis or exclusion of some infectious diseases, some specific types such as tuberculous choroiditis, syphilis after grapes Membrane inflammation, ocular toxoplasmosis, sarcoma-like posterior uveitis, sympathetic ophthalmia, etc., if necessary, carry out some related laboratory tests and auxiliary examinations to confirm the diagnosis; for idiopathic subretinal fibrosis and grapes Membrane syndrome is mainly diagnosed according to the characteristics of fundus lesions and the patient's own characteristics (young women, myopia, no other diseases in the body), fluorescein fundus angiography, indocyanine green angiography, electrophysiology and visual field examination Can provide some help.

Differential diagnosis

Idiopathic subretinal fibrosis and uveitis syndrome should be differentiated from diseases that can cause choroidal, retinal pigment epithelium and cerebral focal lesions in early stages. These diseases can be infectious, such as tuberculous choroiditis, after syphilis Uveitis, diffuse unilateral subacute neuroretinitis, ocular toxoplasmosis, etc.; also non-infectious, such as acute posterior multifocal squamous pigment epithelial lesions, shotgun-like retinal choroidal lesions, multifocal Crohnitis and uvitis, acute retinal pigment epitheliitis, multiple easily dissipative white point syndrome, punctate choroidal colitis (lesion), acute macular retinopathy, sarcoma-like uveitis, pseudo-eye Tissue sporotosis syndrome, etc.; in the later stages of the disease, it should be distinguished from purulent choroiditis and age-related macular degeneration.

1. Subretinal fibrosis and uveitis syndrome

(1) More common in young women, often with myopia.

(2) There is no clear systemic disease.

(3) Sudden vision loss or progressive vision loss.

(4) The incidence of both eyes, but often single eye symptoms are obvious.

(5) Multiple yellow-white lesions (50-500 m) in the posterior pole or middle part of the posterior stage, located at the level of choroid or retinal pigment epithelium.

(6) Later flaky subretinal fibrosis occurs.

(7) may be accompanied by serous retinal detachment, cystoid macular edema, optic disc edema.

(8) may be associated with mild to moderate anterior uveitis, vitreous inflammatory response can be very different in different patients.

(9) Early active fluorescence of active lesions, late staining.

(10) The disease can be recurrent, and most patients have poor visual acuity.

2. Acute posterior multifocal squamous pigment epithelial lesions

(1) There may be a history of flu-like illness or meningeal irritation before onset.

(2) Both eyes have both onset or both eyes, but the interval is short (several days to 2 weeks).

(3) Multiple yellow-white squamous or irregular lesions in the posterior pole, located at the level of retinal pigment epithelium and choroidal capillaries.

(4) The lesion resolves spontaneously within a few days or weeks.

(5) There is no inflammatory reaction or mild to moderate inflammatory reaction in the anterior chamber and vitreous.

(6) The early stage of the lesion showed weak fluorescence, and the late stage showed strong fluorescence.

(7) Most patients have good visual acuity.

3. Acute retinal pigment epitheliitis

(1) The age of onset is too large, mostly over 40 years old.

(2) Visual distortion or central dark spot.

(3) Clustered dark gray lesions clustered in the macular area surrounded by a yellow-white halo.

(4) There may be mild vitreous inflammatory response.

(5) The visual field examination has a central dark spot.

(6) Dark gray spotted lesions showed weak fluorescence, and yellow-white halo showed strong fluorescence.

(7) The lesion disappeared naturally from 6 to 12 weeks.

(8) The visual acuity recovered to a normal level and the prognosis was good.

(9) Generally no recurrence.

4. Claudication choroidal retinitis

(1) The age of onset is too large, 30 to 60 years old.

(2) blue-gray or creamy lesions under the retina with clear borders and multiple adjacent optic discs.

(3) Fresh lesions often appear around old lesions, showing a map-like appearance.

(4) Dark spots in the center or side center.

(5) The active lesions showed weak fluorescence at the early stage, and the edge of the active lesions showed strong fluorescence at the end of the angiography; the inactive lesions showed plaque-like high fluorescence and late staining.

(6) Repeated episodes and progressive progress.

(7) Most patients have poor visual acuity.

5. Shotgun-like retinal choroidal lesions

(1) More common in Caucasians, a rare report of this disease in China.

(2) Generally no systemic performance.

(3) The age of onset is too large, with an average age of 50 years.

(4) bilateral recurrent multiple creamy shotgun-like lesions (50-1500 m) located at the level of choroid and retinal pigment epithelium.

(5) Retinal vasculitis.

(6) Vitreous inflammation without a snowy levee.

(7) cystoid edema of the macula.

(8) A small number of patients may have mild non-granulomatous anterior uveitis.

(9) HLA-A29 antigen is positive.

(10) Early angiography showed weak fluorescence and late angiography.

(11) Recurrent episodes of the disease, some patients have poor visual acuity.

6. Multiple easily dissipative white point syndrome

(1) More common in young women.

(2) Single eye involvement.

(3) Generally no systemic performance.

(4) Multiple yellow-white spotted lesions (100-200 m) located in the deep retina and retinal pigment epithelium.

(5) Granular changes in the macular area.

(6) There is no or only a slight inflammatory reaction in the anterior chamber and vitreous.

(7) Fluorescein fundus angiography revealed strong fluorescence in the early stage of the lesion and late staining.

(8) Indocyanine green angiography revealed multiple weak fluorescent spots.

(9) There is little recurrence of the disease.

(10) The lesions completely resolved within a few weeks to 3 months, and the visual acuity was good.

7. Pointy choroidal lesions

(1) More common in young women.

(2) Both eyes are affected, and the symptoms of one eye are obvious.

(3) Patients have myopia, but generally no systemic disease.

(4) multiple scattered yellow-white fundus lesions (50 ~ 300m), located in the retinal pigment epithelium and inner choroidal level.

(5) There is no anterior uveitis and vitreous inflammatory reaction.

(6) Spontaneous regression of the lesion, leaving a scar of chorioretinal atrophy.

(7) Most patients have good visual acuity, and subretinal neovascularization can affect the visual prognosis of patients.

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