Episcleritis

Eye inflammation in the white of the eye is called scleritis. More superficial inflammation is from episcleritis. Both condtions are usually NOT associated with a recognizable autoimmune disease but scleritis is more likely to be part of a systemic disease. Both are typically easily treatable with steroid eye drops.

Episcleritis is an abrupt onset inflammation in the episclera of one or both eyes, typically presenting as redness, irritation, and watering of the eye with preserved vision. Most patients with episcleritis have a mild, isolated problem that responds readily to topical therapy alone and does not pose a threat to vision. Indeed, many patients with episcleritis require no treatment per se since the condition typically resolves over a short course. A small fraction of patients with episcleritis have an underlying systemic disease that signifies a serious health concern and that requires additional therapy. Episcleritis is only one of many potential causes of a red eye. [1]

Epidemiology

70 percent F>M. Mostly in young and middle-aged adults but may affect all age groups. There are no reliable data regarding the true incidence of episcleritis.  Classified as simple or nodular.

 Simple episcleritis

Simple is usually sectoral, confined to a carefully delimited portion of the episclera, but can also be diffuse, involving the entire surface of the eyeball. 

Simple episcleritis is observed in association with 

 seronegative spondyloarthritides

 inflammatory bowel disease

 forms of vasculitis, particularly those associated with antineutrophil cytoplasmic antibodies (ANCA)

Nodular episcleritis

Nodular  is raised and thus usually limited to just one area of the eyeball. The vast majority of episcleritis cases are isolated and are not associated with a known underlying systemic immune-mediated disorder (unlike scleritis, with which the association is much higher). However, episcleritis may be associated with a number of systemic diseases

Nodular episcleritis is most frequently idiopathic but may be associated with any of the rheumatic conditions, particularly rheumatoid arthritis. Rheumatoid arthritis accounts for approximately 6 percent of all cases of episcleritis, but only a small minority of patients with rheumatoid arthritis develop episcleritis. [1]

Bisphosphonates and episcleritis

Some reports suggest a possible link between use of the bisphosphonate drugs, risedronate and pamidronate, and episcleritis [4,5]. However, a clear cause-and-effect relationship has not been established. Reported occurrence rates are generally less than 1 percent. [1]

Systemic disease

The majority of patients with episcleritis do not have an underlying infectious or systemic inflammatory disease. In a study from a tertiary care medical center, 30 percent of patients had a systemic rheumatic disease and 5 percent had a systemic infection. Among the patients with an underlying systemic condition, the following individual diseases were detected:

Rheumatoid arthritis – 11 percent of the overall cohort

Inflammatory bowel disease – 8 percent

vasculitis – 5 percent

Systemic lupus erythematosus – 3 percent 

Other rheumatic disease – 3 percent

Herpes zoster ophthalmicus – 3 percent

Lyme disease – 3 percent 

 Among community-based populations, the proportion of episcleritis patients who have an underlying systemic inflammatory or infectious condition is probably substantially lower.[1]

Lab testing

Should all patients with episcleritis be referred to rheumatology? Most patients lack any features suggesting an underlying systemic disorder, such as a systemic rheumatic disease, inflammatory bowel disease, or an infectious disorder; thus, consultation and further testing is usually not required. [1]

If systemic disease is suspected consider running the following tests:

CBC, Chemistries, Urinalysis, acute phase reactants

ANCA, RF, CCP, ANA

Differential diagnosis

Uveitis

scleritis

Herpes keratitis

conjunctivitis

Treatment

Episcleritis is not sight-threatening and, in most patients, is an episodic, self-limited process; thus, symptomatic relief should be the goal of therapy. Indeed, many patients with episcleritis require no treatment per se since the condition typically resolves over a short course. For the achievement of symptomatic relief, there are four levels of therapy that we successively employ in the following sequence, depending upon the adequacy of the response to the initial and subsequent intervention: 

Topical lubricants

Topical nonsteroidal antiinflammatory drugs (NSAIDs)

Topical glucocorticoids

Oral NSAIDs

 For some of these treatment modalities, a handful of randomized clinical trials have been performed. [1]

 Initial therapy with topical lubricants

For initial management in patients bothered by their episcleritis, application of topical lubricants is recommended. Any over-the-counter preparation of artificial tears (eg, Refresh Plus or Bion Tears) will suffice, provided it is used four to six times daily. In patients who require more frequent use for symptomatic relief, suggest preparations that do not contain preservatives in order to avoid preservative-induced toxicity. Preservative-free preparations can be used as frequently as required and for as long as the patient remains symptomatic. A small randomized trial found similar effects on the signs and symptoms of episcleritis with artificial tears or topical ketorolac (an NSAID). In this trial, involving treatment of 38 eyes in 37 patients for three weeks, 50 percent improvement in redness and pain was achieved within about four to six days in both groups.

 Topical NSAIDs for persistent discomfort despite lubricants

In patients with significant discomfort, in whom topical lubricants are inadequate, we suggest a topical NSAID (eg, diclofenac ophthalmic drops two to four times daily for several weeks). It is important to exclude dry eyes or other ocular surface epithelial disorders before committing patients to topical NSAIDs, given the potential toxicity of these medications to such eyes. We do not use a topical NSAID as primary therapy, because they are often not needed and can cause ocular toxicity, especially in patients with ocular surface disease, such as dry eyes, who may develop non-healing epithelial defects. 

 Resistant to lubricants and topical NSAIDs

For patients who require therapy beyond topical NSAIDs, employ topical glucocorticoids (eg, fluorometholone acetate [0.1 percent] or prednisolone acetate [1 percent], four times daily). These medications are sufficient in more than 80 percent of cases. It is extremely important to emphasize that chronic glucocorticoid use is associated with a significantly increased risk of cataracts, glaucoma, secondary infection, and corneoscleral thinning. The use of topical glucocorticoids should be directed by an ophthalmologist and should be employed only when the patient remains highly symptomatic despite optimal use of other treatments. A randomized, double-blind clinical trial indicated that fluorometholone acetate (0.1 percent) and prednisolone acetate (1 percent) were comparable in efficacy in the treatment of external ocular inflammation (conjunctivitis, episcleritis, and scleritis). 

 Resistant disease

Oral NSAIDs

In patients who do not respond to topical therapies, or in those with recurrent disease despite such therapy, we suggest an orally administered NSAID. Therapy should be initiated with low doses (eg, naproxen 220 mg twice daily, ibuprofen 400 mg three times daily, or indomethacin 25 mg three times daily). Dose escalation, particularly of indomethacin, should be limited in older patients. These interventions have not been formally compared with other therapies, but in our experience patients usually respond to these medications with improvement in symptoms and findings. 

Oral glucocorticoids

 Rarely, all the above strategies may fail in patients with nodular episcleritis. In such cases, a brief course of oral prednisone starting at 0.5 to 0.75 mg/kg daily with a quick taper over one to two weeks can help the resolution. [1]

Topical

Topical lubricants

Topical nonsteroidal antiinflammatory drugs (NSAIDs)

Topical glucocorticoids

Oral NSAIDs

Systemic

NSAID

Glucocorticoids such as prednisone 0.5 to 0.75 mg/kg daily with a quick taper over one to two weeks [1]