[Scleritis and episcleritis]. / Sclérite et épisclérite.

Scleritis and episcleritis are rare ocular inflammatory diseases but deserve to be known by internists because of their frequent association with systemic autoimmune diseases. It is important to distinguish them between because their prognosis, therapeutic management and potential complications are very different. Episcleritis represents a superficial ocular inflammation with usually benign visual prognosis, no complication with local treatment, and is associated with a systemic autoimmune disease in rare cases. In contrast, scleritis is a potentially serious ophthalmological condition that can threaten the visual prognosis in the absence of appropriate systemic treatment. It is associated with an underlying disease in 40-50% of cases, in particular a systemic autoimmune disease (25-35% of cases) or an infectious cause (5-10% of cases). Rheumatoid arthritis and systemic vasculitides, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, are the main autoimmune causes of scleritis and episcleritis. Scleritis can reveal the underlying autoimmune disease and requires systematic etiological investigations. Aggressive, complicated, refractory forms or those associated with a systemic autoimmune disease require glucocorticoids or even immunosuppressants, and close collaboration between ophthalmologists and internists is required. The development of biologic agents offers new effective therapeutic tools in the management of these difficult cases.

Bilateral nodular episcleritis due to Varicella-Zoster virus / Episclerite nodular bilateral do vírus varicela-zoster

Abstract The patient is a 10 years-old girl, with chief complaint of recent bilateral red eye and history of chickenpox disease. Examination revealed nodular episcleritis of both eyes. After treatment with topical steroids, the disease was subsided. Varicella-Zoster virus is a rare cause of episcleritis. In this case it seems that the mechanism of involvement is immune-related, rather than direct tissue involvement by virus.

Resumo A paciente é uma menina de 10 anos de idade, com queixa principal de olho vermelho bilateral recente e história de doença da catapora. O exame revelou episclerite nodular de ambos os olhos. Após o tratamento com esteróides tópicos, a doença foi diminuída. O vírus varicela-zoster é uma causa rara de episclerite. Neste caso, parece que o mecanismo de envolvimento é imuno-relacionado, ao invés de envolvimento direto do tecido pelo vírus.

[Scleritis and episcleritis]. / Sclérites et épisclérites.

Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus. Among the different types of scleritis, a clear distinction exists between necrotizing forms leading to decreased vision in 50 % of the cases and associated with systemic diseases in the vast majority, and non-necrotizing forms (either diffuse or nodular), with a better prognosis. However, recent publications show that necrotizing forms are much less frequent nowadays (around 5 % of the total), probably due to therapeutic innovations and progress made during the last 20 years. The medical management of scleritis requires collaboration between ophthalmologists and internists (or rheumatologists).

[Scleritis and episcleritis: diagnosis and treatment]. / Sclérites et épisclérites : prise en charge diagnostique et thérapeutique.

Episcleritis and scleritis are distinct entities with regard to visual prognosis, risk of associated systemic disease, and treatment. The pertinence of the clinical classification of episcleritis and scleritis established in 1976 still persists, with significant differences in terms of visual prognosis, associated general conditions, and therapeutic choices according to each scleritis subtype. Episcleritis requires rarely to be referred to a tertiary care centre, and if so it has to be monitored similarly to scleritis. In this paper, an analysis of 1358 scleritis cases from the main distinct large series published since 1976 shows a mean proportion of 8% of infectious aetiologies (mainly herpes viruses), and 28% of systemic diseases with two main subgroups: inflammatory rheumatisms 12.8%, and systemic vasculitis 7.8%. Overall, the risk for visual loss following scleritis is around 16%. However, the risks of associated systemic disease and visual loss are both highly variable according to the type of scleritis, and culminate at 80% and 50% in the necrotizing subtype respectively. As compared with older series, the proportion of necrotizing scleritis is lower in recent series which is likely due to the advances obtained over the past 20 years in immunomodulatory therapy, as well as its wide use in the treatment of the main systemic conditions associated with scleritis. The treatment of scleritis should be managed by physicians who are experts in the use of immunosuppressive drugs that may be required in one out of two affected patients.