Systemic disorders associated with episcleritis and scleritis

Abstract

Inflammation of the episclera and sclera still represents a diagnostic challenge. Many of these presentations are idiopathic and are managed empirically with the use of anti-inflammatory therapy, usually with a satisfactory response. However, some cases will have a more aggressive nature or will show resistance to conventional therapy, and those potentially are connected to an infectious or underlying disease. In such cases, there is an increased risk of visual loss and severe systemic complications requiring intensive investigations and aggressive therapy. The diseases more commonly associated with these conditions, especially with scleritis, are the connective tissue disorders and systemic vasculitis, but systemic infections and malignancies also must be excluded. Management depends on the specific diagnosis, and every year a new list of potential associations or atypical presentations is reported. For a successful outcome, it is important that these presentations are recognized.

Comparative study of ophthalmological and serological manifestations and the therapeutic response of patients with isolated scleritis and scleritis associated with systemic diseases

“…During the therapeutic follow-up of IS patients, the medications used during a crisis were maintained, trying to reduce or even suspend at first the use of systemic corticoids, followed by the immunosuppressors (8,(31)(32)(33)(34) . For the treatment of SASD the aim is to change the initial treatment according to the underlying disease, and in some cases adequate treatment for the systemic disease already improves the ocular disease (4,35) . Alternatively, biological therapies may be used for cases that do not respond to these treatment schemes (36,37) .…”

“…Identifiable causes for scleritis include surgical trauma, bacterial, viral and parasitic infections, autoimmune diseases and primary vasculitis. However, an underlying systemic disease is not identified for the vast majority of cases of scleritis, even after clinical and laboratory tests (4) . Scleritis is greatly associated with systemic rheumatoid disease (30 to 50%), especially rheumatoid arthritis (RA), Wegener's granulomatosis, nodular polyarthritis and systemic lupus erythematosus (SLE) (5)(6)(7) .…”

Esclerite posterior: características clínicas, associação sistêmica, tratamento e evolução de 23 pacientes

“…Interessantemente, em uma pequena série de pacientes (47) analisados em um artigo, houve preponderância de esclerite posterior no sexo masculino e em jovens (média de 19 anos) (13) . Segundo alguns autores, pacientes com mais de 50 anos de idade e esclerite apresentam maior risco de associação com doença sistêmica e perda visual (14)(15)(16) . Também mostram nos trabalhos citados, que não há relação entre o fato de a doença escleral ser unilateral, bilateral e recidivante com a presença de doença sistêmica ou perda visual.…”

“…A maioria dos pacientes do presente trabalho (73,9%) evoluiu com doença unilateral no momento da apresentação. Em 34,8% dos casos, os pacientes apresentaram associação com doença sistêmica concordando com a literatura que afirma um risco entre 30 a 45% de associação entre esclerite posterior e doença sistêmica (16) . Em 62,5%, os pacientes tinham entre 45 a 65 anos de idade.…”

Sclera-Specific and non-sclera-specific autoantibodies in the serum of patients with non-infectious anterior scleritis

“…The abscence of a definite etiologic diagnosis often evokes the possibility of an autoimmune etiology and an immunosuppressive treatment is frequently empirically initiated. Although much research has been devoted to the relationship between scleritis and autoimmunity, the issue is far from being clarified (3) . The detection of auto-antibodies and inflammatory mediators in the serum of patients with scleritis has been taken as evidence of the participation of the immune system.…”