same vasculitic process. Immunosuppressive therapy can be life and eye saving for this group of patients. In patients with suspected (no deÞnitive diagnosis) systemic vasculitic disease and recurrent nodular or diffuse scleritis, the demonstration of inßammatory microangiopathy in conjunctival and/or scleral tissue provides additional justiÞcation for the institution of immunosuppressive therapy.

what probability this ratio favors a speciÞc strategy. The therapeutic plan should reßect an agreement between a well-informed patient and an ophthalmologist who, by virtue of education and training, is an expert in the type of therapy selected and in the early recognition and management of drug-induced complications.

3.3.2Biopsy for Suspected Local

or Systemic Infectious Disease

Detection of an infectious agent in conjunctival and/or scleral tissues is essential in conÞrming a suspected infectious scleritis. Bacteria, fungi, viruses, and parasites may be isolated from conjunctiva and/or scleral tissues of patients with diffuse, nodular, or necrotizing scleritis as a result of direct invasion either from local or systemic infections. Infectious agent isolation in conjunctival and/or scleral tissue allows the institution of speciÞc local or systemic treatment that may improve the ocular and systemic prognoses.

3.6Summary

The approach to the patient with scleritis should include Þve phases. The Þrst phase includes the investigation of the illness through the interview and physical examination of the patient. Because scleritis often portends an underlying, potentially lethal systemic disease, this phase cannot be overemphasized. The interview consists of an analysis of the major complaint and history of the present illness; episcleritis presents as an uncomfortable watery red eye with the absence of severe pain and scleral swelling, whereas scleritis presents with deep, severe, periorbital pain radiating to the head, temple, and jaw, often preventing the patient from sleep. The history continues with the past and family history, the past and present therapy history, and an exhaustive review of systems.

In the fourth phase of the clinical approach to patients with scleritis, the clinical data are integrated with test and biopsy results to conÞrm or discard the preliminary diagnosis. Although clinical data, test results, and biopsy Þndings may not be diagnostic when each is considered independently, the combination of the three can lead to better diagnostic predictions.

3.5Therapeutic Plan

In the Þfth and Þnal phase, therapeutic possibilities for the particular diagnosis are discussed with the patient. Whether considering medical or surgical procedures, the proper role of the doctor is to educate the patient, apprising him or her of the relative risks and beneÞts of possible strategies, and providing an opinion of

as chills, fever, poor appetite, recent weight loss, and fatigue, may suggest a systemic process; skin and hair, respiratory, genitourinary, rheumatological, gastrointestinal, neurological, and ear, nose, and throat manifestations should lead the ophthalmologist to suspect certain types of systemic disorders, such as connective tissue diseases with or without vasculitis, infectious diseases, gout, rosacea, or atopy. Physical examination includes not only the eye, but also the head and extremities; scleral examination includes an external examination in daylight and a slit-lamp examination with white and red-free light. As the ophthalmologist completes this phase, possible diagnoses should come to mind.

The second phase consists of the selection of blood, urine, and imaging studies that are needed to investigate the possibilities raised in the Þrst phase. In the third phase, the decision is made as to whether a tissue biopsy is likely to add useful

information to the diagnosis or to the therapy. The fourth phase integrates the clinical Þndings with tests and biopsy results, leading to a speciÞc diagnosis. In the Þfth phase, a therapeutic plan is initiated and the response is observed.

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Because episcleritis and scleritis are entities encountered infrequently in general ophthalmic practice, the diagnosis and subsequent treatment may be missed or delayed because of the relative lack of experience in their detection and management. Fraunfelder and Watson [1] reported that in a series of 30 enucleated eyes (sent from hospitals from all parts of Great Britain) with a primary histological diagnosis of scleritis, the clinical diagnosis of scleritis had been missed in 12 (40%) often because the scleritis was marked by multiple complications, such as uveitis, glaucoma, and keratolysis. Many of these 12 eyes had not received anti-inßammatory treatment and many of the 18 eyes affected by clinical scleritis had received insufÞcient anti-inßammatory treatment. The main reason for enucleation was pain with loss of vision. Many of these patients had had the ocular inßammation for up to 30 years before the enucleation.

Inßammation of the wall of the eyeball ranges from innocent and harmless self-limiting episcleritis, requiring little or no anti-inßammatory therapy, to very painful, sight-threatening, necrotizing scleritis requiring intensive anti-inßamma- tory and/or immunosuppressive therapy if the globe is to be preserved; moreover, scleritis may often be the presenting manifestation of many potentially lethal systemic diseases. It is, therefore, vital that the correct diagnosis is made and that subsequent adequate treatment is given as early as possible in the course of the disease.

Because of the comparative rarity of scleritis, general considerations on the symptoms, signs,

pathologic Þndings, prognoses, and treatments are difÞcult to obtain. The excellent episcleritis and scleritis survey [2] and treatise [3], performed by Watson and coworkers at MoorÞelds Eye Hospital in London in 1976, has provided us with an exceptional perspective of the spectrum of episcleritis and scleritis. The Scleritis Clinic at MoorÞelds Eye Hospital in London was established in 1963 and since then has been receiving highly selected patients with inßammatory conditions of sclera or episclera. All the patients are referred because of difÞculties in diagnosis, difÞculties in treatment, or both. Subsequent reports by Watson and coworkers on different aspects of diagnosis and management have also been helpful and have served as the foundation on which contemporary studies of smaller patient populations rest [4Ð13]. In an attempt to contribute to the study of the intricacies of scleral disease, we have analyzed our experience with patients with episcleritis and scleritis seen at two tertiary eye care centers, the Massachusetts Eye Research and Surgery Institution (MERSI) in Cambridge, MA, USA, during the 5-year period from April 2005 to April 2010, and the Institute Clinic of Ophthalmology at the Hospital Clinic of Barcelona, Spain, during the 3-year period from April 2007 to April 2010. This study group comprised 585 patients (825 eyes), 85 with episcleritis (119 eyes), and 500 with scleritis (706 eyes) (Table 4.1). The mean follow-up period was 1.77 years (range 1 month to 5 years) for patients with scleritis and 1.43 years (range, 1 month to 5 years) for patients with episcleritis. Our results