Uveitis can be defined as any inflammation affecting the uveal tract, although in clinical practice this term includes any intraocular inflammatory event. The etiology of this inflammation can be related to an endogenous mechanism in the clinical course of a systemic disease (sarcoidosis, Behçet's disease, multiple sclerosis, Vogt-Koyanagi-Harada disease, etc.), or an isolated ocular entity. Sometimes, ocular inflammation is the initial manifestation of an undiagnosed systemic disease. On the other hand, ocular involvement could be the main cause of morbidity of the disease, and early diagnosis and treatment is an important issue in order to avoid irreversible ocular damage.
Uveitis is a group of heterogeneous entities, with very diverse causes, sharing intraocular inflammation as a common denominator. Uveitis are uni- or bilateral and the localization of inflammation defines anterior, intermediate or posterior uveitis. The course of the disease can be chronic or acute. Uveitis are isolated or belong to manifestations of a systemic disease. The identification of a cause of uveitis is based on the analysis of ophthalmic signs and on searching for extraocular manifestations. Laboratory tests should be prescribed according to clinically defined hypotheses. Causes are infectious (bacterial, parasitic, mycotic or viral) or only inflammatory. The prognosis of uveitis is mostly based on their consequences to the macula. When the cause of uveitis is determined, therapeutic guidelines may be applied.
Round 10-12% of all children who present with signs of uveitis suffer from intermediate uveitis. Compared to uveitis anterior in children, the association of intermediate uveitis to a systemic disease is much more complicated. Most cases of uveitis intermedia are idiopathic and show the signs of a pars planitis. Post-infectious and immunological causes are difficult to detect. In cases of uveitis in children, it is necessary to know the specific symptoms of possible underlying systemic diseases. On this basis, an adequate and specific diagnosis will most probably be successful. The term "Masquerade Syndrome" was first used in ophthalmology in 1967 by Theodore, to describe a case of conjunctival carcinoma that manifested as chronic conjunctivitis. The Uveitis Masquerade Syndromes (UMS) are a group of various ocular diseases that may mimic chronic intraocular inflammation. Much malignant pathology may result in an appearance masquerading as uveitis. Uveitis represents a wide spectrum of intraocular inflammatory conditions and includes various autoimmune and infectious etiologies. The relevance of animal models of uveitis to human diseases remains a key issue with major implications for the translational research and development of therapeutic strategies. Histopathological findings in patients with Vogt-Koyanagi-Harada disease, birdshot retinochoroidopathy and anterior uveitis are correlated to those observed in different animal models. Even though evidence based on histopathology is usually irrefutable, similar features may be due to different disease mechanisms. Analysis of triggering factors, determination of cellular populations and immune microenvironment should prevail over clinical phenotype evaluation. There is a controversy in correlating the clinical finding of nummular chorioretinal scars, commonly referred to as Dalen-Fuchs nodules, seen in the periphery of fundus in patients with chronic Vogt-Koyanagi-Harada disease with histologic observations made on such enucleated eyes. Although histopathology of the lesions consisted of focal chorioretinal scars with loss of RPE, there was no consensus about the histologic nature of the nummular chorioretinal scars, particularly whether they represent Dalen-Fuchs nodules. Based on the immunogenetic background, there may be different forms of one specific disease with variable phenotypic expression.
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