Chronic iridocyclitis occurs most often in children of younger age (2-4 years) and almost exclusively in mono-or oligoarthritis variants. When the eye of the special observation sheet is almost 1 / of children with monoartrita has some degree of eye damage when oligoarthritis – is 1/ 6 of the patients. The difficulty of diagnosis lies in the fact that the initial manifestations of chronic iridocyclitis cannot be detected by routine clinical observation, especially since approximately one fifth of the patients with eye lesions precede articular lesions. It should also be noted that, as a rule, the most active iridocyclitis is observed in patients with a small activity of the articular lesion.
A rather variegated ophthalmologic picture of chronic iridocyclitis with YUHA, nevertheless, has a number of specific features that allow a correct diagnosis to be made even without articular syndrome. Specificity is manifested by fragility from vessels, tissue destruction and exudation. One of the most characteristic manifestations is the destruction of the pigment epithelium of the vascular membrane and retina. Probably, this also explains precipitation, proliferation of pigment around synechiae, on a bag of crystals, where the pigmentation reaches such a degree that it resembles a persistent pupillary membrane. The destruction of tissue spreads to the cornea and leads to its characteristic tape-like degeneration. Destruction affects the proper vascular iris, as a result of which its atrophy, thinning, is observedwhich comes to an extreme degree of expression. One of the specific signs is also exudation, which is particularly hard on the vitreous body.
The first clinical signs that appear when the eyes are already grossly damaged are complaints of a child with reduced vision, a feeling of “sand in the eyes”. Objectively, it is possible to detect an enhanced vascular pattern of the eyeball, a change in the shape of the pupil due to synechiae, and hence a poor pupil response to the light of the affected eye.
Approximately one-third of all cases of UXA begin with a poly-arthritic variant. In patients of this group, the most diverse joints are involved in the pathological process, but most often the large joints of the lower extremities. According to the clinical picture, activity and severity of the initial period, this group of patients can be divided into two parts: 1) with a lesion of several large vessels (two thirds of all patients with a polyarthritic onset) and 2) with a lesion of small joints of cyst or STOP.
With the defeat of several large joints, the disease begins, most often, acutely. In the clinical picture, along with a sufficiently bright polyarthritic syndrome (severe pain reaction, changes in the configuration of the joints, impaired function), there are clear signs of intoxication, fever. Laboratory indicators in all cases significantly changed. In these patients, the stable bone of the articular syndrome, its poor curability, and the involvement of other joints and internal organs in the pathological process are noted . In more than half of the patients, in the first years of the disease , bone destructions are recorded, and later, ankylosis is possible (stage 3-4 of X-ray changes according to Sfeinbroker).
When the hands or feet are involved in the pathological process, the initial period often proceeds imperceptibly and the first complaints are: morning stiffness, awkwardness and change in the configuration of the joints. Objectively, proliferative changes in the joints predominate. Most often, this form of lesion is observed in children of school and adolescence. Laboratory activity is rarely observed and never reaches high degrees. In relation to the defeat of the musculoskeletal system, this form of the disease can be considered one of the most
more unfavorable, since within 3-5 years practically all children show gross bone changes (3-4 stages of radiological changes) and changes in the function of the joint (Fig. 8).
Approximately 7-10% of children JCA begins as systems Noah disease. Such a variant of the course is often called the Still syndrome (syndrome). The first and most prominent clinical symptoms are: high hectic temperature, generalized enlargement of lymph nodes, liver, and often spleen. In the initial period, in some patients, it is possible to detect damage to the internal organs: kidneys in the form of glomerulonephritis, lungs – by type of interstitial pneumonia, heart most often myocardium and other organs. Most children have a rash that is diverse in nature, not only in individual patients, but also in one child. In half of the patients at the onset of the disease, the involvement of the articular apparatus is always very bright with a pronounced exudative component. Because of the severity of articular lesions, such children often end up in surgical hospitals with suspected osteomyelitis. In the second half of the patients, the articular syndrome appears later (by 2-3 weeks) from the beginning of the clinical manifestations. In these cases, it is not so pronounced. The disease always occurs with hyperleukocytosis, neutrophilia with a shift to the left, anemia is often observed, ESR is sharply increased, other indicators of the activity of the pathological process are significantly changed.
According to its further course, the Still’s variant is one of the most unfavorable. With adequate therapy, the general manifestations of the disease disappear and articular lesions with persistent, poorly amenable to treatment, rapid, compared with other forms, bone destruction and disability of patients by the locomotor system, come to the fore. In half of the patients, a lesion of the internal organs and, above all, of the kidneys, is found. Basically, this formidable complication of UHA as amyloidosis is further formed in this group of children. ,
A special form of Yuha is a subsepsis of Wissler-Fanconi. The attribution of this form to Yuha and to this day is debatable, since at least half of the children with further observation does not reveal chronic arthritis. However, the similarity of its symptoms with the initial manifestations of other forms of UHA, in some patients, the development of typical chronic arthritis allows us to consider allergic subsepsis as a form or, more precisely, as a phase in the development of UHA.
In most cases, the disease begins acutely. The most typical clinical signs are: temperature reaction, rash, pain in the joints, and often – visceral symptoms. The temperature is usually high, the wrong type, there are frequent rises in the morning and night hours. It is noteworthy that children usually tolerate temperature without disturbing their well-being. Rash is one of the main symptoms for the disease and is observed in all children. Most patients have a rash with polymorphism; it is usually maculopapular, less often urticular, macular or anular. Localization of the rash is different, most often – on the limbs, trunk, rarely – on the face, and sometimes only over the affected joints. Most children have complaints of pain in joints by type of polyarthralgia.Objective changes in the joints in the initial period of the disease are rarely observed.
Of the visceral manifestations, heart failure in the form of myocarditis or myopericarditis is most often observed . Approximately half of the patients have changes in the urine in
form of transient hematuria and proteinuria. The defeat of other internal organs is rare.
Peripheral blood in the period of acute events is characterized by leukocytosis, not rarely — hyperleukocytosis, neutrophilia with a shift to the left, significantly increased ESR. Other indicators of activity (proteinogram, sialic acids, etc.) are also significantly changed.
As already noted, approximately half of the patients (in recent years, 70%) of sub sepsis are acute and end with recovery after one or two acute periods. In the rest of the children, the pathological process is localized in the joints and the disease acquires the typical features of YUHA, mostly in the form of a polyarthric form. It can be noted that if at the beginning of the disease the pain in the joints is localized or such localization ). appears during re-activation, the risk of developing chronic arthritis in these children is significantly higher than in patients with intermittent arthralgia.
As already mentioned, in the case of UHA at the onset of the disease, and especially significant in the course of the evolution of the pathological process, a lesion of the internal organs is observed. Most significantly for
‘prognosis of the disease eye and kidney damage. The first has already been mentioned above. The frequency of kidney damage varies widely depending on the amount of research methods . On biopsy and sectional material, changes are found in a third of children in the presence of glomerulonephritis and interstitial nephritis, often with morphological signs of amyloidosis. In the Initial Stages, kidney pathology manifests as moderate hematuria and proteinuria without impairment of function. Stable and expressed proteinuria in a patient with YUHA is always suspicious of the development of amyloidosis.
Often the heart is involved in the pathological process . Most often, a heart lesion manifests itself in the form of myocarditis, a valve organ lesion described by some authors has not been encountered even once. At the height of exacerbations with pronounced general activity, pericarditis can be involved in the pathological process; exudative pericarditis passes quickly with a decrease in activity. Much less frequently than the heart and kidneys, the lungs are affected in the form of vascular lesions, less often – pleurisy. Involvement in the pathological process of the liver in UHA is less common, and more often, changes on the part of this organ are not associated with the disease itself, but with the therapy being conducted. The latter circumstance must be taken into account when diagnosing lesions of other organs, since long-term therapy with anti-inflammatory drugs, and especially hormonal and cytostatic,can lead to pronounced dystrophic changes
nenyami from the heart, kidneys, liver and other organs. In conclusion of the clinical section, I would like to cite data on the frequency of different variants of the initial period and different forms of YUHA after a long observation (the results are based on the observation of 500 children in the initial period of the disease and 200 children with follow-up monitoring of more than 5 years LPMI).
Such special forms, which are found and well studied in adults with RA, do not occur as Felty’s symptom complex with YUHA, and Sjogren’s syndrome (Sjogren) is observed as a casuistry (Tables 1 and 2).