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 scleritis. The spectrum of systemic diseases1 accompanied by eye damage is extremely wide, but mainly these are diseases of the rheumatological category. In addition, according to the international and domestic classification of rheumatic diseases, this group includes systemic diseases of non-rheumatic genesis.

Systemic diseases associated 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, a distinction is made between anterior (like iridocyclitis), peripheral (involving the flat part and periphery of the retina of the ciliary body), posterior (retinovasculitis, neurochorioretinitis) uveitis and panuveitis (involving all parts of the eye).

Depending on the nature of the course, there are acute, subacute, chronic and recurrent lesions.

Based on the etiological features, uveitis in systemic diseases in children and adolescents is classified according to the type of systemic disease.

Juvenile rheumatoid arthritis

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

Uveitis, often bilateral (up to 80%), is outwardly asymptomatic, which leads to late diagnosis. As a rule, uveitis has the character of anterior, iridocystitis, but less characteristic peripheral and panuveitis also occur.

In the absence of preventive examinations by an ophthalmologist, the process in the eye is often detected at the stage of development of complications (lectoid dystrophy, pupillary closure, complicated cataract) with a significant decrease in vision.

Cyclitis may be accompanied not only by hypotony and the formation of corneal precipitates, but also by exudation into the vitreous body of varying intensity, which leads to the development of floating and semi-fixed opacities in the vitreous body. The posterior segment of the eye is very rarely involved in the process, but signs of papillitis, small yellowish foci on the middle periphery of the fundus, and cystic macular degeneration are possible.

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

Seronegative spondyloarthritis

Juvenile ankylosing spondylitis

Juvenile ankylosing spondylitis is a chronic inflammatory disease characterized by a combination of uveitis and peripheral arthritis. It is rarely recognized at its onset.

Mostly boys under 15 years of age are affected. However, many cases of early onset are known. Uveitis usually proceeds as acute bilateral and relatively benign (like anterior uveitis, quickly relieved by local treatment). Acute iritis in juvenile ankylosing spondylitis may be accompanied by pain, photophobia, and redness of the eyes. Changes in the posterior segment of the eye are rare.

Most patients are seropositive for HLA-B27 and seronegative for RF.

Juvenile psoriatic arthritis

Uveitis in psoriatic arthritis is clinically similar to rheumatoid arthritis, and occurs as anterior, chronic or acute. The course is often favorable and responds well to standard treatment. It should be emphasized that uveitis usually occurs in patients with a combination of skin and joint lesions and is almost never seen in isolated skin psoriasis.

Reiter's syndrome (urethrooculosynovial syndrome)

Reiter's syndrome with eye damage in children is relatively rare. Children aged 5-12 years may get sick, the development of the process is more typical in teenagers and young men aged 19-40 years.

Eye diseases in Reiter's syndrome usually occur in the form of conjunctivitis or subepithelial keratitis with damage to the anterior stromal layers. Recurrent nongranulomatous iridocyclitis may develop. Much less frequently, uveitis may have the character of severe panuveitis with the development of complicated cataracts, macular edema, chorioretinal foci, retinal detachment, and a sharp decrease in vision.

Systemic lupus erythematosus

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

Eye damage may occur as episcleritis or scleritis. More typical is the development of severe diffuse retinovasculitis with occlusion of the central retinal vein and other vessels, retinal edema, hemorrhages, and in severe cases, recurrent hemophthalmos.

Behcet's disease

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

Uveitis in Behcet's disease is bilateral and can occur as either anterior or posterior uveitis. It most often develops 2-3 years after the first signs of the disease appear. It should be noted that uveitis in Behcet's disease is recurrent, with exacerbations lasting from 2-4 weeks to several months.

A distinctive feature of uveitis in Behcet's disease is hypopyon of varying severity in a third of patients. In anterior uveitis, the prognosis for vision is mostly favorable. In panuveitis and involvement of the posterior part of the eye, severe complications develop, leading to irreversible changes in the eyes (atrophy of the optic nerve, dystrophy, retinal detachment, recurrent hemophthalmos). The presence of retinovasculitis with occlusion of retinal vessels is characteristic.

Sarcoidosis

Sarcoidosis is a chronic multisystem granulomatous disease of unknown etiology.

In children over 8-10 years old, sarcoidosis occurs with characteristic lesions of the lungs, skin and eyes, at the age of under 5 years, a triad is noted - 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 few symptoms. But it is severe, like chronic granulomatous anterior uveitis, with the formation of large "fatty" precipitates and nodules on the iris. When the posterior segment of the eye is involved in the process, small yellowish foci 18-14 RD (choroidal granulomas), periphlebitis, and macular edema are detected on the fundus. Rapid development of complications with decreased vision is characteristic.

Vogt-Koyanagi-Harada syndrome (uveameningeal syndrome)

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

The diagnosis is based on the identification of a symptom complex: changes in the skin and hair - alopecia, poliosis, vitiligo; mild neurological symptoms (paresthesia, headache, etc.). In the form of severe uveameningitis in children, the disease rarely occurs.

Uveitis in Vogt-Koyanagi-Harada syndrome occurs as bilateral severe granulomatous chronic or recurrent panuveitis with the formation of numerous large precipitates, nodules in the iris and its thickening, neovascularization of the iris and the angle of the anterior chamber, which leads to an increase in intraocular pressure. Vitritis phenomena are expressed. Edema of the optic nerve head, macula, sometimes exudative retinal detachment in the central zone are characteristic. Yellowish foci with clear boundaries are detected on the periphery (more often in the lower section). Complications such as secondary glaucoma quickly develop, vitreous membranes appear. The prognosis for vision is unfavorable.

Lyme-Borreliosis

Lyme borreliosis is transmitted by the bite of ixodid ticks infected with Borrelia burgdorferi and occurs in stages:

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

Eye damage can be of various nature, occurring as conjunctivitis, episcleritis, anterior uveitis. Retinovasculitis or panuveitis occur much less frequently. Uveitis responds well to treatment, the prognosis for vision is usually favorable.

CINCA syndrome

CINCA (chronic infantile neurologic cutaneous articular) syndrome is characterized by migratory skin changes (urticarial), arthropathy with epiphyseal and meta-epiphyseal changes, CNS lesions, deafness, and hoarseness.

Uveitis develops in 90% of patients with CINCA syndrome, proceeds mainly as anterior, less often posterior uveitis, but with the rapid development of complicated cataract. Papillitis and optic nerve atrophy are characteristic, leading to a significant decrease in vision.

Cogan's syndrome

Cogan's syndrome is accompanied by fever, arthritis, abdominal pain, hepatosplenomegaly, lymphadenopathy. Hearing loss, dizziness, ringing and pain in the ears are noted, aortitis, aortic valve insufficiency, vasculitis of small and medium-sized arteries develop. If the eyes are involved, interstitial keratitis, conjunctivitis, episcleritis/scleritis, and less commonly, vitriitis, retinovasculitis occur.

Kawasaki disease

Characteristic signs of Kawasaki disease are fever, cutaneous vasculitis (polymorphic rash), "raspberry" tongue, peeling; lymphadenopathy, hearing loss. Eye damage is mainly of the type of bilateral acute conjunctivitis. Much less common is uveitis, which is mild, asymptomatic, anterior bilateral, more often in children over 2 years of age.

Wegener's granulomatosis

Wegener's granulomatosis is characterized by fever, arthritis, damage to the upper respiratory tract, lungs; stomatitis, otitis, cutaneous vasculitis; kidney damage. Eye damage is characterized by conjunctivitis, episcleritis, less often - keratitis with the formation of a corneal ulcer. Uveitis occurs as a retinovasculitis.

Drug treatment

A distinction is made between local and systemic drug treatment.

Local treatment includes the use of glucocorticoids, NSAIDs, mydriatics, etc. Its intensity and duration 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, eye medicinal films, parabulbar injections, phono- and electrophoresis. For parabulbar or subconjunctival administration, prolonged drugs can be used, which reduces the number of injections.

If there is no positive effect from local drug therapy and in severe uveitis, systemic treatment is prescribed (in conjunction with a rheumatologist).

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

Data on the effectiveness and appropriateness of using TNF-a inhibitors in uveitis are highly contradictory. Infliximab (Remicade) is a biological agent, soluble antibodies to TNF-a. It is used intravenously by drip at 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 to TNF-a. It is used at a dose of 0.4 mg/kg or 25 mg subcutaneously 2 times a week.

Recently, both rheumatologists and ophthalmologists have been giving preference not to monotherapy with one of the drugs, but to their combination in lower dosages.

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

This is why it is necessary to always remember the possible dissociation of the severity and expression of the ocular and systemic disease, when the decision on the tactics of systemic treatment presents great difficulties.

Surgical treatment

Considering the high severity of uveitis in systemic diseases in children with the development of a wide range of complications, surgical interventions play an important role in the treatment of uveitis:

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

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