Frost dendritic retinal vasculitis
Introduction
Introduction to frosty dendritic vasculitis Frosted dendritic vasculitis (frostedbranchangiitis) is a retinal perivascular inflammation that is a rare type of uveitis. Mainly involving the vein, the artery is rare. Healthy adolescents are predominant, 10 years old and 20-30 years old are the two peaks of their incidence, and women account for 2/3. The characteristic fundus of the disease is changed to a white remnant of the retinal blood vessels, which forms a white sheath around the blood vessels, like a branch covered with frost, hence the name. Most of the vascular involvement is obvious in the middle and the periphery. A few of the posterior poles are dominant, and both the artery and the vein can be involved, but the venous involvement is more obvious and serious. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: cataract glaucoma retinal detachment
Cause
Cause of frosty dendritic vasculitis
(1) Causes of the disease
It is not entirely clear that the current speculation may be related to the following factors:
Immune response factors (30%):
The disease is typically characterized by extensive retinal vasculitis and perivascular inflammation, which responds well to glucocorticoids and can be combined with autoimmune diseases such as systemic lupus erythematosus, Vogt-Koyanagi Harada syndrome, Crohn's disease, etc. Therefore, some people think that immune factors play a certain role in its occurrence. The release of immune complexes in local deposition and the production of complement active products may be one of the important mechanisms of its pathogenesis.
Low immune function (32%):
This disease occurs mostly in children and children with low immune function (such as acquired immunodeficiency syndrome), suggesting that the reduction of immune function may play a role in the occurrence of this disease; 4 tumors, large cell lymphoma and leukemia have been found Patients may have frosty dendritic vasculitis, and some believe that the infiltration of tumor cells can lead to the disease.
Infection factor (25%):
It has been found to be associated with infection with a variety of viruses (such as cytomegalovirus, human immunodeficiency virus, Epstein-Barr virus, herpes simplex virus, etc.), Toxoplasma infection.
(two) pathogenesis
The pathogenesis of this disease is not fully understood.
Prevention
Frosty dendritic vasculitis prevention
The onset of vasculitis should avoid strenuous exercise, standing for a long time and sitting for a long time, each time should not exceed half an hour, so as to avoid the formation of lower extremity edema, more exudation, should raise the affected limb. If a vasculitis patient has to stand and sit for a long time due to work, he should be active for half an hour or lie down and lift his feet for three minutes.
Complication
Frosty dendritic vasculitis complications Complications cataract glaucoma retinal detachment
Patients with acute iridocyclitis can completely resolve, very few patients with post-iris adhesions, complicated cataracts, secondary glaucoma and other complications; vitreous inflammation can be completely absorbed, a few mechanization, causing posterior vitreous detachment; There are macular stenosis, fibrous scars, narrowing of retinal blood vessels, focal or even map-like atrophy of the retina, secondary retinal detachment, branch retinal vein occlusion, retinal neovascularization, retina Complications such as bleeding, these complications can be seen in the special hair style and systemic type, but more common in the whole body type.
Symptom
Creamy dendritic vasculitis symptoms common symptoms fundus changes eye shadow ciliary congestion retinal hemorrhagic photophobia
According to whether it is accompanied by systemic diseases, frost-like dendritic vasculitis can be divided into two types: one type is unexplained, the fundus has characteristic changes, and no systemic diseases are common. It is more common in oriental children, on glucocorticoids. Hormone therapy is sensitive, usually does not recur after healing, some people call it a special hair style; the other type is a certain cause, the fundus performance is more complicated, and there are many kinds of fundus changes in the defrost-like dendritic vasculitis. Combined with systemic diseases, it is more common in Westerners. In addition to glucocorticoid therapy, it is still necessary to treat the cause. This type of complication is more and more difficult to cure. It may recur due to the recurrence of systemic diseases. This type is named, we will call it the whole body type.
1. Ocular performance: Sudden onset of the patient, usually complained of sudden redness, blurred vision or decreased vision, may have photophobia, black shadow and other symptoms, the degree of visual decline may be very different, some patients may Obvious vision decreased, but most patients have severely reduced vision, and even reduced to light, especially for both eyes, and single eye involvement is very rare.
Normal or mild to moderate iridocyclitis in the anterior segment of the eye, manifested as ciliary congestion, dusty or linear corneal depression, anterior chamber flash, anterior chamber inflammatory cells, vitreous light to moderate dusty or fog A turbidity, afferent pupillary disorder has been reported, which may be due to severe exudative detachment of the macula or retinal detachment.
The characteristic fundus of this disease is changed to a white remnant of the retinal blood vessels around the blood vessels. White sheaths are formed around the blood vessels, like branches covered with frost. Therefore, the name of the blood vessels is more obvious in the middle and the periphery, and a few are mainly in the middle. Both the movement and the vein can be affected, but the venous involvement is more obvious and serious. Therefore, Kleiner et al. pointed out that the name "acute frost-like retinal vein inflammation" may better reflect the clinical manifestations of the disease.
The special hair style is different from other systemic type of fundus. The former retina has extensive edema or local exudation, especially in the peripheral part. The posterior pole retina is extensive or scattered in punctate or flaky, and the optic disc is normal. Congestion, edema, etc., retinal vascular tortuosity and dilation, cystoid edema, exudative detachment, but no macular hole, but also obvious vitreous hemorrhage, central retinal vein occlusion, etc. In addition to the characteristic changes in the fundus of the patient, there may be lesions consistent with the primary disease, such as changes in typical cytomegalovirus retinitis, local retinal necrosis and exudative lesions, which may have a bit or flaky hemorrhage. Retinal cotton plaques caused by human immunodeficiency virus infection may occur, and frosty dendritic vasculitis associated with systemic lupus erythematosus may cause exudative macular detachment, scattered intraretinal hemorrhage and a "white heart" appearance Retinal hemorrhage (ie, there is a white lesion in the center of the bleeding).
2. Systemic manifestations: Special hair types mainly occur in healthy people, and there are no incentives. However, there are also reports of colds, viral conjunctivitis, skin acne rashes, etc. 1 to 5 weeks before the onset of eye diseases. Some patients may have antibodies against pathogens, such as antibodies against herpes simplex virus, herpes zoster virus, Epstein-Barr virus, Streptococcus, etc. Patients with AIDS may be diagnosed with human immunodeficiency virus infection several years before eye disease, and combined Opportunistic infections (such as Pneumocystis, oral Candida albicans, Pneumocystis carinii, cytomegalovirus, etc.), Kaposi sarcoma can still occur, but also after the occurrence of frosty dendritic vasculitis Defective virus infection, therefore, serological retinal vasculitis in adults should be serological and other tests to determine the cause or combined systemic disease, in addition to patients with acute glomerulonephritis, sterilized Meningitis and other diseases.
Examine
Examination of frosty dendritic vasculitis
For a small number of suspicious patients, especially those with systemic diseases, anti-human immunodeficiency virus, cytomegalovirus, herpes simplex virus, Toxoplasma gondii and other antibodies should be tested, and immune function and systemic examination should be performed to determine their association. Systemic disease.
Fluorescein fundus angiography can detect delayed filling of the affected arteries, extensive retinal fluorescein leakage, cystoid macular edema, optic disc staining, hemorrhage obscuration, capillary perfusion, retinal neovascularization, etc.; indocyanine green angiography Weak fluorescent spots and choroidal vascular dye leakage can be found; electroretinogram shows that both rod and cone function are significantly inhibited, showing a, b wave amplitude decreasing, oscillation potential disappearing, retinal current map and pattern visual evoked potential The change can completely return to normal after the disease is cured; the visual field examination can find changes such as widening of the visual field and expansion of the physiological blind spot.
Diagnosis
Diagnosis and identification of frosty dendritic vasculitis
According to typical eye changes, especially fundus changes, it is generally not difficult to make a diagnosis.
Differential diagnosis
1. Acute retinal necrosis syndrome: This disease is usually characterized by retinal necrosis of the middle part of the retina, retinal vasculitis mainly composed of retinal arteritis, significant vitreous opacity and late-stage rhegmatogenous retinal detachment. Herpes simplex virus, herpes simplex virus infection, according to the characteristics and clinical manifestations of fluorescein fundus angiography, it is generally not difficult to distinguish it from frost-like dendritic vasculitis.
2. Intermediate uveitis: The middle uveitis is typically characterized by ciliary body flat and vitreous base-like snow-like lesions, spheroidal opacity in the vitreous, often associated with peripheral retinal vasculitis, retinal choroiditis, and macular cystic edema Such changes do not occur in a wide range of retinal vascular sheaths, these clinical features help to identify the two.
3. Cytomegalovirus or herpes simplex retinitis: Viral infection may be the cause or associated disease of systemic frosty dendritic vasculitis, and more often associated with AIDS, therefore, when suspicious patients are found, it should be detailed Systemic examinations and laboratory tests to rule out the presence of such serious illnesses.
4.Eales disease: Eales disease involves the peripheral retinal vein branch, the wall is accompanied by white sheath or opacity, but the vascular sheath is not as extensive as that caused by frosty dendritic vasculitis, and repeated massive blood in the vitreous is Its characteristics are easy to distinguish from frost-like dendritic vasculitis.
