Thread: scleritis

scleritis

Background
Scleritis is an inflammatory disease that affects the sclera; it may be localized, nodular, or diffuse. It may involve the anterior (visible segment) and/or posterior segments of the eye and manifests with redness of the eye and severe eye pain. Isolated posterior scleritis will not present with redness of the visible portion of the eye and may or may not present with pain.

The 4 types of anterior scleritis are as follows:
1. Diffuse anterior scleritis: This is characterized by widespread inflammation of the anterior portion of the sclera. This is the most common form of anterior scleritis as well as the most benign.
2. Nodular anterior scleritis: This type is characterized by one or more erythematous, immovable, tender inflamed nodules on the anterior sclera. Approximately 20% of cases progress to necrotizing scleritis.
3. Necrotizing anterior scleritis with inflammation: This form frequently accompanies serious systemic collagen vascular disorders including rheumatoid arthritis. Pain with this condition is usually extreme, and damage to the sclera is often marked. Necrotizing anterior scleritis with corneal inflammation is also known as sclerokeratitis.
4. Necrotizing anterior scleritis without inflammation: This type most frequently occurs in patients with long-standing rheumatoid arthritis; it is due to the formation of a rheumatoid nodule in the sclera and is notable for its absence of symptoms. Necrotizing anterior scleritis without inflammation is also known as scleromalacia perforans.
Necrotizing anterior scleritis is the most severe form and most common form of scleritis with vision-threatening complications and resultant permanent visual loss.

Posterior scleritis occurs much less frequently than anterior scleritis, but they may occur concurrently.

In cases of non-necrotizing scleritis, vision is often maintained unless complications such as uveitis occur.

The examiner must be cognizant of conditions that masquerade as scleritis such as toxoplasmosis-induced posterior uveitis and chronic lymphocytic leukemia. Episcleritis often presents similarly to scleritis, but inflammation and erythema is isolated to the episclera, which lies between the sclera and the conjunctiva. Episcleritis has a more benign course and does not cause any visual changes or permanent impairment; however, recurrence is common.

Pathophysiology
The sclera, which consists of collagen and elastic connective tissue, provides a tough protective casing around the eye. Enzymatic degradation of collagen fibrils and invasion of inflammatory cells, including T cells and macrophages, appear to play an important role.

The thickness of the sclera varies from 0.3-1.2 mm. Healthy sclera is consistently white. Inflammation, the principal pathology affecting the sclera, is frequently part of a general inflammatory reaction associated with a systemic immune-mediated collagen vascular disease.

Inflammation of the sclera can progress to ischemia and necrosis, eventually leading to scleral thinning and perforation of the globe. Necrotizing anterior scleritis represents a particularly destructive form of scleritis.
Frequency
United States
Scleritis is an uncommon disease. Well-defined incidence rates are hard to find. The prevalence is estimated to be 6 cases per 10,000 population. Of patients diagnosed with scleritis, anterior scleritis is demonstrated in 94% of patients, as opposed to posterior scleritis, which is diagnosed only 6% of the time.

An increased incidence of scleritis has been reported in patients taking bisphosphonates, which are commonly used in the management of osteoporosis.
International
No particular geographic distribution has been noted.
Mortality/Morbidity
• Morbidity arises from primary scleritis and associated systemic disease.
• Giant pigment epithelial tear and retinal detachment has been reported in a patient with scleritis.
• A significant percentage of patients with concurrent scleritis and collagen vascular disease die within 5 years.
• In 15% of cases, scleritis is the presenting manifestation of collagen vascular disorder and may precede additional symptoms by one to several months.
Race
No published information is available on racial differences.
Sex
The female-to-male ratio is approximately 1.6:1.
Age
• Cases have been reported in patients ranging from 11-87 years of age. Mean age for all types of scleritis is 52 years.
• Episcleritis tends to affect younger patients

Clinical
History
• Pain
• Severe, constant, deep, boring, or pulsating pain is noted.
• Pain worsens with movement of the eye.
• Pain is worse at night and may awaken the patient.
• Pain may be referred to the eyebrow, temple, or jaw. Occasionally, the configuration of the pain pattern corresponds to the course of the trigeminal nerve.
• Onset: Scleritis is subacute, a more gradual onset occurs than is seen in episcleritis.
• Duration of symptoms: Scleritis generally persists from months to years, whereas episcleritis usually resolves within weeks.
• Associated symptoms
• Erythema of the eye is a defining symptom with anterior scleritis.
• Lacrimation
• Photophobia
• Discharge is ordinarily not part of the clinical picture of scleritis.
• Less prevalent symptoms
• Fever
• Nausea/vomiting
• Headache
• Unilateral or bilateral: Both eyes are affected in slightly more than one half of cases.
• Similar previous episodes
• Prior history
• Underlying systemic disease
• Trauma
• Ocular surgery
• Glaucoma
• Exposure to irritants/chemicals
• Previous use of eye drops
• Medications
Physical
• As with any eye complaint (except chemical injury), begin with vision testing.
• Visual acuity may be normal or decreased with all forms of scleritis.
• Visual impairment is most pronounced with posterior scleritis.
• Additionally, a complete physical examination, particularly of the skin, joints, heart, and lungs, may be obligatory when an underlying complicating illness is suspected.
• External examination
• Ensure that the eye examined is supported by satisfactory lighting, inspecting for breadth and degree of injection, as well as the presence of bluish hue signifying attenuation of the sclera.
• Prominent findings may include photophobia, tearing without discharge, tenderness of the eye, and purplish red, edematous, engorged blood vessels.
• Deeper sclera blood vessels appear darker, follow a radial pattern, and do not move when manipulated with a cotton swab.
• Administration of topical phenylephrine 2.5-10% causes blanching of the more superficial episcleral vessels but does not change the engorgement of deeper sclera vessels and can help differentiate between scleritis and episcleritis.
• Perform a slit lamp/biomicroscopic examination to judge the depth and breadth of involvement.
• Determine whether there is diffuse or segmental involvement. Widespread injection of the conjunctival and deep scleral vessels is characteristic of diffuse anterior scleritis.
• Localized elevation of the sclera is representative of nodular anterior scleritis.
• Nodules in anterior scleritis are immobile, differing from the non-mobile nodules that can be seen in episcleritis.
• Scleral thinning is suggested when the choroid pigment becomes a blue-violet hue best seen in natural lighting.
• Global perforation typically results in an abnormal shape of the pupil and/or uveal or vitreous prolapse.
• Use of a red-free filter (green light) helps identify avascular areas of the sclera. Corneal changes are present in up to 50% of cases.
• Examination of the eyelids for possible blepharitis or conjunctivitis should be performed.
• Assessment of the anterior chamber for possible narrowing, hyphema, or hypopyon. Angle narrowing can be seen in acute angle-closure glaucoma. Hyphema can be seen with trauma and/or bleeding disorders. Hypopyon may be seen in uveitis/iritis.
• Posterior scleritis
• External findings associated with posterior scleritis include restriction of eye movements, sensitivity to palpation, and proptosis.
• Dilation of the fundus may be necessary to identify posterior scleritis. Posterior scleritis may simulate amelanotic choroidal.
• Funduscopic examination of the patient with posterior scleritis may also reveal papilledema, choroidal folds, and retinal hemorrhage or detachment.

Causes
Scleritis coexists with a serious systemic disease in almost one half of cases; the underlying problem is frequently a connective tissue disorder.
• Rheumatoid arthritis is the underlying disease for approximately one sixth of patients suffering from scleritis, and approximately 1% of patients with rheumatoid arthritis will develop scleritis at some point in the course of the disease. Scleritis associated with RA is due to the development of a rheumatoid nodule on the sclera and is associated with an increased risk of mortality.
• Other connective tissue and autoimmune diseases seen with scleritis include the following:
• Systemic lupus erythematosus (SLE)
• Polyarteritis nodosa
• Seronegative spondyloarthropathies
• Ankylosing spondylitis
• Psoriatic arthritis
• Reactive arthritis
• Wegener granulomatosis
• Relapsing polychondritis
• Sarcoidosis
• Inflammatory bowel disease
• Sjörgen syndrome
• Additional maladies that may accompany scleritis include the following:
• Syphilis
• Post herpes zoster ophthalmicus
• Tuberculosis
• Gout
• Lyme disease
• Foreign body
• Hypertension

Differential Diagnoses
Conjunctivitis, Allergic
Keratoconjunctivitis, Atopic

Conjunctivitis, Bacterial
Keratoconjunctivitis, Epidemic

Conjunctivitis, Giant Papillary
Keratoconjunctivitis, Sicca

Conjunctivitis, Viral
Keratoconjunctivitis, Superior Limbic

Episcleritis
Pterygium

Glaucoma, Angle Closure, Acute
Toxoplasmosis

Keratitis, Bacterial
Trigeminal Neuralgia

Keratitis, Fungal
Uveitis, Classification

Keratitis, Herpes Simplex

Keratitis, Interstitial

Other Problems to Be Considered
Cerebral tumor
Chronic lymphocytic leukemia
Immunosuppression
Collagen vascular disease

Workup
Laboratory Studies
• Depending on the clinical suspicion, laboratory studies may be warranted. Laboratory tests include, but are not limited to, the following:
o Complete blood count (CBC) and electrolytes
o Erythrocyte sedimentation rate (ESR)
o FTA-ABS (RPR)
o Uric acid
o Rheumatoid factor
o Antinuclear antibody (ANA)
• The ED is the ideal location to initiate evaluation for collagen vascular disease, infection (conjunctival cultures), and immunocompetency. If scleritis is suspected, the emergency physician will contact an ophthalmologist. This would be a good opportunity to establish an evaluation and intervention plan. Getting the laboratory studies in the ED will save time for the patient and practitioners.
Imaging Studies
• B-scan ultrasonography may assist in detecting posterior scleritis. MRI or CT scans may play a role, but they should be ordered in consultation with an ophthalmologist.
• Chest radiography may be indicated to look for underlying pulmonary involvement arising from systemic disease.
• Imaging of sacroiliac joints is prudent when ankylosing spondylitis is suspected.
Other Tests
• Instillation of phenylephrine, a mydriatic vasoconstrictor, helps differentiate deep scleral episcleral blood vessel involvement from superficial involvement; superficial vessels blanch following application of phenylephrine, while deeper vessels remain unaffected.
• Seidel test helps detect possible global perforation. Apply a moistened fluorescein strip over the potential site of perforation while viewing under a slit lamp. If perforation exists, the fluorescein dye becomes diluted by the aqueous humor, appearing as a green dilute stream within the dark, concentrated, orange dye.
Procedures
• Apply an eye shield when scleral thinning or global perforation is suspected.
o Ensure that the eye guard does not contact the eyelid or globe.
o Apply tape from the forehead to zygoma.
o In the absence of an eye shield, use a paper or polystyrene cup, provided it is large enough to cover the eye without placing undue pressure on the globe.

Medication
Therapeutic goals for scleritis are familiar to any emergency practitioner: relieve the patient’s pain and initiate therapy that will positively alter the course of the disease.

From the patient's perspective, pain cessation may be the most important action taken by the emergency practitioner. Outcome will depend on the patient's response to immunosuppressive therapy.

Narcotics and systemic NSAIDs may render temporary pain relief and can be started in the ED.

NSAIDs are generally found to be effective in approximately one third of patients with diffuse anterior scleritis and two thirds of patients with nodular anterior scleritis. NSAIDs have also been found to be helpful in patients with idiopathic posterior scleritis.

Initiation of immunosuppressive therapy may require coordination with an internist and/or rheumatologist.

Topical steroids have a high failure rate but should be discussed with the practitioner who will provide follow-up care.

Subconjunctival steroid injections for non-necrotizing scleritis remain controversial. Localized steroid injections may lead to increased intraocular pressure, scleral melting, or globe perforation/scleral rupture.

Surgical management is generally not required except in rare cases of necrotizing scleritis.
Nonsteroidal anti-inflammatory drugs
These agents are used to decrease pain and inflammation. NSAIDs are thought to act by inhibiting prostaglandin synthesis, interfering with migration of leukocytes, and inhibiting phosphodiesterase.

Indomethacin (Indocin)
Often considered the DOC. Indomethacin is rapidly absorbed. Metabolism occurs in the liver by demethylation, deacetylation, and glucuronide conjugation.
Adult
25-50 mg/dose IR PO bid/tid
75 mg SR PO bid; not to exceed 200 mg/d
Pediatric
1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists); perform ophthalmologic studies in patients who develop eye complaints during therapy, and discontinue therapy if changes (eg, blurred or diminished vision, corneal deposits and retinal disturbances, scotomata, changes in color vision, macula degeneration) noted
Diflunisal (Dolobid)
Nonsteroidal salicylic acid derivative that acts peripherally as an analgesic. Has antipyretic and anti-inflammatory effects; however, differs chemically from aspirin and is not metabolized to salicylic acid. It is a prostaglandin-synthetase inhibitor
Adult
Initial: 500-1000 mg PO
Maintenance: 250-500 mg PO divided bid; not to exceed 1.5 g/d
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetusD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
At high doses, diflunisal inhibits platelet function and bleeding time; ophthalmologic examinations should be performed in patients who develop eye complaints during treatment; peripheral edema has been reported with diflunisal use; caution when in cardiovascular conditions that predispose patient to fluid retention; because diflunisal is a salicylic acid derivative, may potentially cause Reye syndrome
Naproxen (Naprelan, Anaprox, Aleve, Naprosyn)
Used for relief of mild-to-moderate pain. It inhibits inflammatory reactions and pain by decreasing the activity of the enzyme cyclooxygenase, resulting in a decrease of prostaglandin synthesis.
Naproxen is rapidly absorbed and has a half-life of 12-15 h. It is highly protein bound.
Adult
375-500 mg PO bid
Pediatric
<2 years: Not established
>2 years: 2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug
Ibuprofen (Motrin, Ibuprin, Advil)
Usually the DOC for treatment of mild- to- moderate pain, if no contraindications exist. Inhibits inflammatory reactions and pain, probably by decreasing activity of the enzyme cyclooxygenase, which results in prostaglandin synthesis.
Highly protein-bound drug that is readily absorbed orally. The half-life is short (1.8-2.6 h).
Adult
400-600 mg PO qid
Pediatric
<6 months: Not established
6 months to 12 years: 10-70 mg/kg/d PO divided tid/qid; start at lower end of dosing range and titrate to maximum of 2.4 g/d
>12 years: Administer as in adults
Sulindac (Clinoril)
Decreases activity of cyclooxygenase and, in turn, inhibits prostaglandin synthesis. Results in a decreased formation of inflammatory mediators.
Adult
150-200 mg PO bid or 300-400 PO qd; not to exceed 400 mg/d
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetusD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in preexisting renal disease or compromised renal perfusion; low white blood cell counts occur rarely, and usually return to normal in ongoing therapy; discontinuation of therapy may be necessary if leukopenia, granulocytopenia, or thrombocytopenia persists; caution in anticoagulation defects or in those who are receiving anticoagulant therapy
Piroxicam (Feldene)
Chemically different from other NSAIDs. Extensively bound to plasma proteins. Decreases activity of cyclooxygenase and, in turn, inhibits prostaglandin synthesis. These effects decrease formation of inflammatory mediators.
Adult
10-20 mg/d PO qd
Pediatric
0.2-0.3 mg/kg/d PO qd; not to exceed 15 mg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists)
Immunosuppressive agents
These agents are used in severe (necrotizing scleritis) and resistant forms of the disease. Only an ophthalmologist experienced with the medication should prescribe these drugs.

Methotrexate (Folex, Rheumatex)
Mechanism of action in treatment of inflammatory reactions is unknown. May affect immune function and usually ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Adult
7.5-15 mg PO qwk or 15 mg IM qwk
Pediatric
5-15 mg/m2/wk PO/IM as a single dose or as 3 divided doses given 12 h apart
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBCs monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs including salicylates has not been tested)
Cyclophosphamide (Cytoxan, Neosar)
Chemically related to nitrogen mustards. As it is an alkylating agent, mechanism of action of active metabolites may involve cross-linking of the DNA, which may interfere with growth of normal and neoplastic cells.
Adult
1-2 mg/kg/d PO
Pediatric
Administer as in adults
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis
Azathioprine (Imuran)
Inhibits mitosis and cellular metabolism by antagonizing purine metabolism and inhibiting synthesis of DNA, RNA, and proteins.
Adult
1 mg/kg/d PO/IV for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d
Pediatric
Initial: 2-5 mg/kg/d PO/IV
Maintenance: 1-2 mg/kg/d PO/IV
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur
Cyclosporine (Sandimmune, Neoral)
Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs.
For children and adults, dosing should be based on ideal body weight.
Adult
3-5 mg/kg PO qd
Pediatric
Administer as in adults
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO
Glucocorticoids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli and are useful in the treatment of recurrent scleritis.

Methylprednisolone (Depo-Medrol, Solu-Medrol, Medrol)
Administered IM or IV. Usually used in addition with other immunosuppressive agents.
Adult
1 g IV 3 times/wk
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use; Depo-Medrol contains benzyl alcohol, which is potentially toxic when administered locally to neural tissue
Administration of Depo-Medrol by other than indicated routes, including the epidural route, has been associated with reports of serious medical events including arachnoiditis, meningitis, paraparesis/paraplegia, sensory disturbances, bowel/bladder dysfunction, seizures, visual impairment including blindness, ocular, and periocular inflammation, and residue or slough at injection site
Prednisone (Deltasone, Orasone, Sterapred)
Used to treat inflammatory and allergic reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.
Adult
60-120 mg PO qd; following satisfactory response, taper over 2-3 wk to approximately 20 mg/d with gradual taper (2.5-mg increments) until discontinuation
Pediatric
2 mg/kg PO qd
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Medication
Therapeutic goals for scleritis are familiar to any emergency practitioner: relieve the patient’s pain and initiate therapy that will positively alter the course of the disease.

From the patient's perspective, pain cessation may be the most important action taken by the emergency practitioner. Outcome will depend on the patient's response to immunosuppressive therapy.

Narcotics and systemic NSAIDs may render temporary pain relief and can be started in the ED.

NSAIDs are generally found to be effective in approximately one third of patients with diffuse anterior scleritis and two thirds of patients with nodular anterior scleritis. NSAIDs have also been found to be helpful in patients with idiopathic posterior scleritis.

Initiation of immunosuppressive therapy may require coordination with an internist and/or rheumatologist.

Topical steroids have a high failure rate but should be discussed with the practitioner who will provide follow-up care.

Subconjunctival steroid injections for non-necrotizing scleritis remain controversial. Localized steroid injections may lead to increased intraocular pressure, scleral melting, or globe perforation/scleral rupture.

Surgical management is generally not required except in rare cases of necrotizing scleritis.
Nonsteroidal anti-inflammatory drugs
These agents are used to decrease pain and inflammation. NSAIDs are thought to act by inhibiting prostaglandin synthesis, interfering with migration of leukocytes, and inhibiting phosphodiesterase.

Indomethacin (Indocin)
Often considered the DOC. Indomethacin is rapidly absorbed. Metabolism occurs in the liver by demethylation, deacetylation, and glucuronide conjugation.
Adult
25-50 mg/dose IR PO bid/tid
75 mg SR PO bid; not to exceed 200 mg/d
Pediatric
1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists); perform ophthalmologic studies in patients who develop eye complaints during therapy, and discontinue therapy if changes (eg, blurred or diminished vision, corneal deposits and retinal disturbances, scotomata, changes in color vision, macula degeneration) noted
Diflunisal (Dolobid)
Nonsteroidal salicylic acid derivative that acts peripherally as an analgesic. Has antipyretic and anti-inflammatory effects; however, differs chemically from aspirin and is not metabolized to salicylic acid. It is a prostaglandin-synthetase inhibitor
Adult
Initial: 500-1000 mg PO
Maintenance: 250-500 mg PO divided bid; not to exceed 1.5 g/d
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetusD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
At high doses, diflunisal inhibits platelet function and bleeding time; ophthalmologic examinations should be performed in patients who develop eye complaints during treatment; peripheral edema has been reported with diflunisal use; caution when in cardiovascular conditions that predispose patient to fluid retention; because diflunisal is a salicylic acid derivative, may potentially cause Reye syndrome
Naproxen (Naprelan, Anaprox, Aleve, Naprosyn)
Used for relief of mild-to-moderate pain. It inhibits inflammatory reactions and pain by decreasing the activity of the enzyme cyclooxygenase, resulting in a decrease of prostaglandin synthesis.
Naproxen is rapidly absorbed and has a half-life of 12-15 h. It is highly protein bound.
Adult
375-500 mg PO bid
Pediatric
<2 years: Not established
>2 years: 2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug
Ibuprofen (Motrin, Ibuprin, Advil)
Usually the DOC for treatment of mild- to- moderate pain, if no contraindications exist. Inhibits inflammatory reactions and pain, probably by decreasing activity of the enzyme cyclooxygenase, which results in prostaglandin synthesis.
Highly protein-bound drug that is readily absorbed orally. The half-life is short (1.8-2.6 h).
Adult
400-600 mg PO qid
Pediatric
<6 months: Not established
6 months to 12 years: 10-70 mg/kg/d PO divided tid/qid; start at lower end of dosing range and titrate to maximum of 2.4 g/d
>12 years: Administer as in adults
Sulindac (Clinoril)
Decreases activity of cyclooxygenase and, in turn, inhibits prostaglandin synthesis. Results in a decreased formation of inflammatory mediators.
Adult
150-200 mg PO bid or 300-400 PO qd; not to exceed 400 mg/d
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetusD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in preexisting renal disease or compromised renal perfusion; low white blood cell counts occur rarely, and usually return to normal in ongoing therapy; discontinuation of therapy may be necessary if leukopenia, granulocytopenia, or thrombocytopenia persists; caution in anticoagulation defects or in those who are receiving anticoagulant therapy
Piroxicam (Feldene)
Chemically different from other NSAIDs. Extensively bound to plasma proteins. Decreases activity of cyclooxygenase and, in turn, inhibits prostaglandin synthesis. These effects decrease formation of inflammatory mediators.
Adult
10-20 mg/d PO qd
Pediatric
0.2-0.3 mg/kg/d PO qd; not to exceed 15 mg/d
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animalsD - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists)
Immunosuppressive agents
These agents are used in severe (necrotizing scleritis) and resistant forms of the disease. Only an ophthalmologist experienced with the medication should prescribe these drugs.

Methotrexate (Folex, Rheumatex)
Mechanism of action in treatment of inflammatory reactions is unknown. May affect immune function and usually ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Adult
7.5-15 mg PO qwk or 15 mg IM qwk
Pediatric
5-15 mg/m2/wk PO/IM as a single dose or as 3 divided doses given 12 h apart
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBCs monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs including salicylates has not been tested)
Cyclophosphamide (Cytoxan, Neosar)
Chemically related to nitrogen mustards. As it is an alkylating agent, mechanism of action of active metabolites may involve cross-linking of the DNA, which may interfere with growth of normal and neoplastic cells.
Adult
1-2 mg/kg/d PO
Pediatric
Administer as in adults
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis
Azathioprine (Imuran)
Inhibits mitosis and cellular metabolism by antagonizing purine metabolism and inhibiting synthesis of DNA, RNA, and proteins.
Adult
1 mg/kg/d PO/IV for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d
Pediatric
Initial: 2-5 mg/kg/d PO/IV
Maintenance: 1-2 mg/kg/d PO/IV
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur
Cyclosporine (Sandimmune, Neoral)
Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs.
For children and adults, dosing should be based on ideal body weight.
Adult
3-5 mg/kg PO qd
Pediatric
Administer as in adults
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO
Glucocorticoids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli and are useful in the treatment of recurrent scleritis.

Methylprednisolone (Depo-Medrol, Solu-Medrol, Medrol)
Administered IM or IV. Usually used in addition with other immunosuppressive agents.
Adult
1 g IV 3 times/wk
Pediatric
Not established
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use; Depo-Medrol contains benzyl alcohol, which is potentially toxic when administered locally to neural tissue
Administration of Depo-Medrol by other than indicated routes, including the epidural route, has been associated with reports of serious medical events including arachnoiditis, meningitis, paraparesis/paraplegia, sensory disturbances, bowel/bladder dysfunction, seizures, visual impairment including blindness, ocular, and periocular inflammation, and residue or slough at injection site
Prednisone (Deltasone, Orasone, Sterapred)
Used to treat inflammatory and allergic reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.
Adult
60-120 mg PO qd; following satisfactory response, taper over 2-3 wk to approximately 20 mg/d with gradual taper (2.5-mg increments) until discontinuation
Pediatric
2 mg/kg PO qd
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

thanks........