Table 3.1 Clinical classiÞcation of episcleral and scleral inßammation

Episcleritis

Simple

Nodular

Scleritis

Anterior scleritis Diffuse scleritis Nodular scleritis Necrotizing scleritis:

With inßammation

Without inßammation (scleromalacia perforans) Posterior scleritis

Adapted from reference [1]

Fig. 3.1 Episcleritis. Note the vascular dilatation of conjunctival vessels, and superÞcial episcleral and deep episcleral vascular plexuses. There is no underlying scleral edema or loss of sclera, and the eye appears bright red

Fig. 3.2 Scleritis. Note the bluish-red appearance of the inßamed eye, owing to the loss of some of the scleral Þbers under the conjunctiva and episcleral tissue

Table 3.2 Diseases associated with episcleritis and scleritis

Noninfectious

Connective tissue diseases and other inßammatory conditions

Rheumatoid arthritis Systemic lupus erythematosus Ankylosing spondylitis Reactive arthritis

Psoriatic arthritis

Arthritis and inßammatory bowel disease Relapsing polychondritis

Vasculitic diseases Polyarteritis nodosa

Allergic granulomatous angiitis (ChurgÐStrauss syndrome)

Granulomatosis with polyangiitis (Wegener) Beh•etÕs disease

Giant-cell arteritis CoganÕs syndrome

Vasculitic diseases associated with connective tissue diseases and other inßammatory conditions

Miscellaneous

Atopy

Rosacea

Gout

Foreign body granuloma

Chemical injury

Infectious

Bacterial

Fungal

Viral

Parasitical

therapeutic possibilities for the particular diagnosis are presented to the patient and, after appropriate discussion of the options, a therapeutic plan is initiated and the response to therapy observed. Each step in this simpliÞed model of the clinical approach to scleral disease can be analyzed individually.

3.1Investigation of the Illness

The Þrst phase consists of the interview and the physical examination of the patient. During the interview, the ophthalmologist evaluates the major complaint, characteristics of the present illness, past and family diseases, and past and present therapies; a review of systems is made as well. The physical examination is made not only of the eye, but also of the head and extremities.

Table 3.3 Phases of clinical approach to episcleritis and scleritis

1. Investigation of the illness Interview

Major complaint History of present illness Past history

Family history

Past and present therapy history Review of systems

Physical examination Systemic examination

Head Extremities

Ocular examination Episclera and sclera Other ocular structures

2. Diagnostic tests Blood and urine tests

Anterior chamber polymerase chain reaction (PCR) Smears and cultures (if infection is suspected) Skin testing

Imaging studies

3. Tissue biopsy

4. Integration of clinical Þndings with tests and biopsy results

5. Therapeutic plan

Assessment of different therapeutic possibilities Discussion of risks and beneÞts with patient

3.1.1Major Complaint and History of Present Illness

The major complaint is the main problem that motivated the patient to seek medical help. The ophthalmologist must begin with an open-ended question to allow the patient to freely describe the major complaint and the history of the present illness. Even though this may result in a disjointed and incomplete story, inßections of voice, facial expression, descriptive efforts with hands, and attitude may betray important clues to the meaning of what the patient has been experiencing. The ophthalmologist should actively guide the interview in order to develop organization and content, to clarify terms the patient uses, and to evaluate symptoms as of little or considerable importance. Severity of symptoms may be estimated by the extent to which they interfere with sleep or work patterns. The patient soon learns that events must be dated, sequences established, and onset and characteristics of symptoms precisely described.

The main symptoms present in scleral diseases are the following.

1.Pain is the most common symptom for which patients with scleral disorders seek medical assistance, and is the best indicator of the presence of active inßammation. Pain is due to both direct stimulation and stretching of the nerve endings by the inßammation. Important issues to consider in pain evaluation are the patientÕs age, cultural background, environment, and psychological circumstances, such as depression, anxiety, and tension. Inquiry should be made concerning character, location, radiation, timing, and analgesic response. No pain, mild discomfort, or occasional trivial pain localized to the eye is characteristic of episcleritis. ÒRealÓ pain, severe, penetrating pain that radiates to the forehead, the brow, the jaw, or the sinuses, awakens the patient during the night, is only temporarily relieved by analgesics, is characteristic of scleritis. We place great emphasis on pain in discriminating between episcleritis and scleritis. Differential diagnosis of other painful eye diseases, including corneal surface problems, angle-closure glaucoma, and acute anterior uveitis, are easily excluded by ocular examination. Differential diagnosis of other painful periocular diseases, including migraine, sinusitis, herpes zoster ophthalmicus, and orbital tumors, are excluded by careful ocular and periocular examination, and additional imaging studies.

2.Redness, a sign rather than a symptom, can be detected by the patientÕs family or friends or by the patient looking in the mirror. Almost all patients with episcleritis and scleritis present to the physician with redness as a clinical manifestation. Clinically obvious redness may be absent in scleromalacia perforans (necrotizing scleritis without inßammation) and in posterior scleritis.

3.Tearing or photophobia without mucopurulent discharge occurs in approximately one-fourth of patients with episcleral and scleral inßammation, but it is usually mild or moderate.

4.Tenderness to palpation may be described by the patient. In general, ocular tenderness is mild, if any, and localized to the site of inßammation in episcleritis or is moderate or severe and diffuse, with possible radiation to other parts of the head in true scleritis.

ing in scleritis. The extension of the scleral inßammation to the adjacent structures may cause keratitis, uveitis, glaucoma, papillitis, macular edema, annular ciliochoroidal detachment, and serous retinal detachment; these abnormalities may impair visual acuity.

3.1.2Past History

The past medical and ocular history elicitation serves primarily to discover already known systemic diseases that can cause scleritis (Table 3.2). Past ocular surgical procedures, especially in the few months prior to the onset of scleritis, can have potential diagnostic importance. Determination of past medical history is also important for discovering certain conditions, such as gastric ulceration, diabetes, liver or renal disease, or hypertension that might eventually modify future therapy.

3.1.3Family History

The family history can be important in several respects. The growing Þeld of immunogenetics has demonstrated that diseases frequently associated with scleritis, such as rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, reactive arthritis (we moved from ÒReiter«s diseaseÓ term because Reiter was a criminal as a consequence of doing experimentations on prisoners in concentration camps during World War 2), BehcetÕs disease, atopy, and gout, have a genetic basis. This information may give clues for speciÞc systemic diagnoses that can be pursued with additional studies. Also, psychological factors, such as attitudes toward illness, fears, and expectations, can be assessed from previous illnesses within the family.

3.1.4Past and Present Therapy History

The history of past and present therapies and response to them is extremely important. Type of therapy, route of administration, dosage, response,

tion often provide insight about the nature of the illness and for future therapeutic decision making. Careful attention to detail may often reveal simple, correctable mistakes, such as inadequate dosage; poor compliance, too short a trial of a slow-acting medication; medications given for other conditions; or foods that interfere with medications prescribed for the scleritis.

There is a great variability in patient response to nonsteroidal anti-inßammatory drugs. Sometimes, one nonsteroidal anti-inßammatory drug may be better than another for a particular patient; therefore, inadequate response to one nonsteroidal anti-inßammatory drug does not indicate probable unresponsiveness to other nonsteroidal anti-inßammatory drugs. Furthermore, at least 2Ð4 weeks are required for full evaluation of a particular nonsteroidal anti-inßammatory agent. Therefore, caution is indicated in drawing conclusions from the therapeutic response to only one type of nonsteroidal anti-inßammatory drug or from responses to nonsteroidal anti-inßamma- tory drugs used for brief periods.

The most important features of the steroid therapy history include the length of therapy, route of administration, maximal and average doses, side effects, and whether periodic attempts to decrease the dose were made (and if so, the size of the decrements). This historical information may be essential in deciding whether to initiate immunosuppressive therapy.

The patient should be asked whether he or she has ever used immunosuppressive drugs; type, dosage, response, and side effects are extremely important to future treatment planning.

3.1.5Review of Systems

Because episcleritis and especially scleritis can be associated with systemic disorders, a detailed inquiry into the different systems of the body must be made. Systemic signs or symptoms may lead the ophthalmologist to suspect certain types of disorders, such as connective tissue diseases with or without vasculitis, atopy, rosacea, gout,

Table 3.4 Review of system questionnaire for episcleritis and scleritis

Table 3.4 (continued)

Gastrointestinal

SLE systemic lupus erythematosus, RA rheumatoid arthritis, RP relapsing polychondritis, PAN polyarteritis nodosa, GPA (Weg) granulomatosis with polyangiitis (Wegener), Ch-S allergic granulomatous angiitis (ChurgÐStrauss syndrome), GCA giant-cell arteritis, Reactive A reactive arthritis, PA psoriatic arthritis, AS ankylosing spondilitis, IBD arthritis associated with inßammatory bowel disease, VZV varicella zoster virus (herpes zoster), Syph syphilis, TB tuberculosis, Ros rosacea, Lyme Lyme disease, Behçet Beh•et«s disease, Cogan CoganÕs syndrome