Eye damage in children and adolescents with systemic diseases: causes, symptoms, diagnosis, treatment

Eye damage in children and adolescents with systemic and syndromic diseases occurs in 2-82% of cases and includes mainly uveitis and sclerites. The spectrum of systemic diseases, accompanied by eye lesions, is extremely wide, but mostly it is a disease of the rheumatological heading. In addition, according to the international and domestic classification of rheumatic diseases, this group includes systemic diseases of non-rheumatic genesis.

Systemic diseases, combined with eye damage

• Juvenile rheumatoid arthritis.
• Juvenile ankylosing spondylitis.
• Juvenile psoriatic arthritis.
• Reiter's syndrome.
• Behcet's disease.
• Vogt-Koyanagi-Harada Syndrome.
• Sarcoidosis.
• Systemic lupus erythematosus.
• Lyme disease.
• Rare syndromes (CINCA, Kogan, etc.).

Most systemic diseases with eye damage include arthritis and arthropathies, so this group includes reactive arthritis with eye damage, as well as some rare syndromes.

Classification

Based on the clinical picture, the anterior (by type of iridocyclitis), the peripheral (with the involvement of the flat part and the periphery of the retina of the ciliary body), the posterior (retinovasculitis, neurochorioretinitis) uveitis and panoveitis (involving all parts of the eye).

By the nature of the current, acute, subacute, chronic and recurrent lesions are distinguished.

On the etiologic sign of uveitis in systemic diseases in children and adolescents are classified according to the type of systemic disease.

Juvenile rheumatoid arthritis

The frequency of uveitis in juvenile rheumatoid arthritis varies from 6 to 18%, reaching 78% for mono- and oligoarthritis. In most cases (up to 86.6%) uveitis precedes joint diseases.

Uveitis, more often bilateral (up to 80%), is externally asymptomatic, which leads to late diagnosis. As a rule, the uveitis has the character of the anterior, iridoclitic, but there are also less characteristic peripheral and panoveites.

The process in the eye in the absence of preventive examinations by an ophthalmologist is more often detected in the stage of development of complications (lectotic dystrophy, pupillary infection, complicated cataract) with a significant decrease in vision.

Cyclitis can accompany not only hypotension and the formation of corneal precipitates, but also exudation into the vitreous humor of varying intensity, which leads to the development in the vitreous body of floating and semi-fixed opacities. The posterior part of the eye is involved in the process very rarely, however, signs of papillitis, small yellowish foci on the middle periphery of the fundus, and racemose maculodystrophy are possible.

When organizing preventive examinations, the ophthalmologist should remember that the risk factors for uveitis in juvenile rheumatoid arthritis are mono- or oligoarthritis, female patients, early debut of arthritis, and the presence of an antinuclear factor. Uveitis can occur at different times from the onset of a systemic disease, therefore systematic ophthalmologist examinations are necessary (every 6 months in the absence of complaints).

Seronegative spondylitis

Juvenile ankylosing spondylitis

Juvenile ankylosing spondylitis is a chronic inflammatory disease characterized by a combination of uveitis with peripheral arthritis. It is rarely recognized in the debut.

Mostly boys under the age of 15 are ill. However, many cases of early onset are known. Uveitis usually proceeds as acute bilateral and relatively benign (according to the type of anterior uveitis, it is quickly stopped with local treatment). Acute iritis with juvenile ankylosing spondylitis can accompany pain, photophobia, redness of the eyes. Changing the posterior segment of the eye is rare.

Most of the patients are seropositive according to HLA-B27 and seronegative for RF.

Juvenile psoriatic arthritis

Uveitis with psoriatic arthritis is clinically similar to rheumatoid, proceeds according to the type of anterior, chronic or acute. The course is often favorable, well suited to standard treatment. It should be emphasized that uveitis, as a rule, occurs in patients with a combination of skin and joint damage and almost does not occur with isolated skin psoriasis.

Reiter's syndrome (urethrookulosinovial syndrome)

Reiter's syndrome with eye damage in children is relatively rare. Children at the age of 5-12 years can be ill, more typical development of the process in adolescents and young men 19-40 years.

Eye diseases with Reiter's syndrome, as a rule, occur in the form of conjunctivitis or subepithelial keratitis with damage to the anterior layers of the stroma. Possible development of recurrent non-granulomatous iridocyclitis. Significantly less often uveitis can be of the nature of severe panoveitis with the development of complicated cataracts, macular edema, chorioretinal foci, retinal detachment, a sharp decrease in vision.

Systemic lupus erythematosus

Systemic lupus erythematosus is a multi-system autoimmune disease with skin lesions (rashes, including on the face in the form of a "butterfly"); joints (non-erosive arthritis); kidneys (nephritis); heart and lungs (serous membranes).

Eye damage can occur as episcleritis or scleritis. More characteristic is the development of a severe retinovasculitis of a diffuse nature with occlusion of the central vein of the retina and other vessels, retinal edema, hemorrhage, and in severe cases, recurrent hemophthalmia.

Behcet's disease

Eye damage in Behcet's disease is one of the most severe and prognostically unfavorable manifestations.

Uveitis with Behcet's disease is bilateral and can proceed both in the type of anterior and posterior uveitis. Most often it develops 2-3 years after the first signs of the disease. It should be noted that uveitis in Behcet's disease is recurrent, with the duration of exacerbations ranging from 2-4 weeks to several months.

A distinctive sign of uveitis in Behcet's disease is hypopion of varying severity in a third of patients. With anterior obliquity, the prognosis for vision is predominantly favorable. With panoveitis and involvement in the process of the posterior eye, severe complications develop, leading to irreversible changes in the eyes (atrophy of the optic nerve, dystrophy, retinal detachment, recurrent hemophthalmus). Characterized by the presence of retinovasculitis with occlusion of retinal vessels.

Sarcoidosis

Sarcoidosis is a chronic multi-system granulomatous disease of unclear etiology.

In children older than 8-10 years, sarcoidosis occurs with a characteristic lesion of the lungs, skin and eyes, at the age younger than 5 years, note the triad - uveitis, arthropathy, rash. It should be noted that arthritis in sarcoidosis occurs only in children, in adults the joints are not involved in the pathological process.

Uveitis, as a rule, proceeds externally with little sign. But it is difficult, according to the type of chronic granulomatous anterior uveitis, with the formation of large "sebaceous" precipitates and the formation of nodules on the iris. When involved in the process of the posterior segment, the eye on the fundus reveals small yellowish foci of 1 / 8-1 / 4 RD (choroidal granulomas), the phenomenon of periphoebitis, macular edema. Characteristic is the rapid development of complications with reduced vision.

Vogt-Koyanagi-Harada Syndrome (uveamenegal syndrome)

Vogt-Koyanagi-Harada syndrome is a systemic disease involving auditory and visual analyzers, skin and meninges.

The diagnosis is based on the identification of the symptom complex: changes in the skin and hair - alopecia, polyosis, vitiligo; mild neurological simtomatika (parasthesia, headache, etc.). In the form of severe uveameningitis in children, the disease is rare.

Uveitis with Vogt-Koyanagi-Harada syndrome proceeds as a bilateral, heavy granulomatous chronic or recurrent panoveitis with the formation of numerous large precipitates, nodules in the iris and its thickening, neovascularisation of the iris and the anterior chamber angle, which leads to an increase in intraocular pressure. The phenomena of vitriate are expressed. Characteristics of edema of the optic nerve, macula, and sometimes exudative retinal detachment in the central zone. On the periphery (more often in the lower section) yellowish foci with clear boundaries are identified. Rapidly developing complications of the type of secondary glaucoma, vitreal membranes arise. The prognosis for vision is unfavorable.

Lime-Borreliosis

Lyme borreliosis (Lyme borreliosis) is transmitted by biting ixodid ticks infected with Borrelia burgdorferi, proceeds in stages:

• Stage I - migratory annular erythema, regional lymphadenopathy;
• II stage - hematogenous dissemination in various organs, including the eyes and joints.

The defeat of the eyes can be of a varied nature, flowing like conjunctivitis, episcleritis, anterior uveitis. Significantly, retinovascular or panoveitis occurs. Uveitis responds well to treatment, a vision prognosis is usually favorable.

CINCA Syndrome

For the syndrome of CINCA (chronic infantile neurologic cutaneous articular) migratory skin changes (urticaria), arthropathy with epiphyseal and metaepiphysis changes, CNS lesions, deafness, hoarseness are characteristic.

Uveitis develops in 90% of patients with CINCA syndrome, mainly of the anterior type, less often of the posterior uveitis, but with the rapid development of complicated cataracts. Characteristic of papillitis, atrophy of the optic nerve, leading to a significant decrease in vision.

Cogan's syndrome

Cogan's syndrome is accompanied by fever, arthritis, abdominal pain, hepatosplenomegaly, lymphadenopathy. There is a decrease in hearing, dizziness, ringing and pain in the ears, aortitis, aortic valve failure, vasculitis of small and medium-sized arteries. When involved in the eye process, there is interstitial keratitis, conjunctivitis, episcleritis / scleritis, and rarely - vitriate. Retinovascular.

Kawasaki disease

Typical signs of Kawasaki's disease are fever, skin vasculitis (polymorphic rash), "crimson" tongue, peeling; lymphadenopathy, hearing loss. Eye defeat mainly as a bilateral acute conjunctivitis. Significantly, there is uveitis, which proceeds gently, asymptomatically as anterior bilateral, more often in children older than 2 years.

Wegener's granulomatosis

Typical for Wegener's granulomatosis are fever, arthritis, upper respiratory tract infection, lung; stomatitis, otitis media, cutaneous vasculitis; kidney damage. For eye involvement, conjunctivitis, episcleritis, and rarely keratitis with the formation of corneal ulcers are characteristic. Uveitis proceeds according to the type of retinovascular.

Medication

There are local and systemic medication.

Local treatment includes the use of glucocorticoids, NSAIDs, mydriatics, etc. The intensity and duration of it varies and depends on the severity of the eye disease. The leading role in local treatment is played by glucocorticoid drugs, which are used in the form of drops, ointments, ophthalmic medicinal films, parabulbar injections, phono and electrophoresis. For the introduction of parabulbar or under the conjunctiva, it is possible to use prolonged drugs, which allows reducing the number of injections.

In the absence of a positive effect on the use of local medication and in case of severe uveitis, systemic treatment is prescribed (together with a rheumatologist).

The most effective in the treatment of uveitis against the background of systemic diseases are glucocorticoids, metrotrescate and cyclosporin A. These drugs are often used in various combinations, which allows to reduce the working dose of each of them.

Data on the efficacy and appropriateness of the use of TNF-a inhibitors in uveitis are highly controversial. Infliximab (remicade) is a biological agent, soluble antibodies to TNF-a. Apply intravenously drip in a dose of 3-5 mg / kg: one administration at intervals of 2-6 weeks every 8 weeks.

Etanercept (enbrel) is a biological agent, soluble receptors for TNF-a. Apply in a dose of 0.4 mg / kg or 25 mg subcutaneously 2 times a week.

Recently, both rheumatologists and ophthalmologists prefer not to monotherapy with one of the drugs, but their combination at lower dosages.

It should be emphasized that rather often prescribed systemic treatment, having a pronounced positive effect on the course of the underlying disease, does not allow us to stop uveitis. Moreover, often a particularly severe course of eye disease is noted during the remission of the joint (systemic) process, when rheumatologists sharply reduce the intensity of treatment, leaving only maintenance doses of drugs or limiting the use of NSAIDs.

That is why it is always necessary to remember the possible dissociation of the severity and severity of the ocular and systemic disease, when the solution of the question of the tactics of systemic treatment presents great difficulties.

Surgery

Given the great severity of the course of uveitis in systemic diseases in children with the development of a wide range of complications, an important place in the treatment of uveitis is occupied by surgical interventions:

• surgical treatment in violation of the transparency of optical media (cornea, lens, vitreous body); cataract removal, vitrectomy;
• antiglaucomatous interventions of various types;
• treatment of secondary retinal detachments (laser and instrumental).

[1], [2], [3], [4], [5], [6]