Sie sind auf Seite 1von 18

Scleritis

Practice Essentials

Scleral inflammation (scleritis) may occur in one or both eyes. Pain is a


hallmark symptom.

Signs of scleritis include focal or diffuse redness or violaceous


discoloration, initial scleral thickening, late scleral thinning, nodules, and
necrosis. It may be associated with keratitis, iritis, glaucoma, and exudative
retinal detachment.

Scleritis commonly has an underlying cause, usually an autoimmune


disease (rheumatoid arthritis, granulomatosis with polyangiitis, other
vasculitic/connective tissue diseases).

Treatment include corticosteroids, nonsteroidal anti-inflammatory drugs


(NSAIDs), immunosuppressives, and biologics.

Background

Scleritis is a chronic, painful, and potentially blinding inflammatory disease


that is characterized by edema and cellular infiltration of the scleral and
episcleral tissues (outermost coat of the eye).

Scleritis may be isolated to the eye, but is commonly associated with


systemic autoimmune disorders, including rheumatoid arthritis, systemic
lupus erythematosus, relapsing
polychondritis, spondyloarthropathies, Wegener
granulomatosis, polyarteritis nodosa, and giant cell arteritis.

The correct and rapid diagnosis and the appropriate systemic therapy can
halt the relentless progression of both ocular and systemic processes, thus
preventing destruction of the globe while prolonging survival and improving
quality of life.

Scleritis may be classified into anterior and posterior. Anterior scleritis can
be diffuse, nodular, necrotizing with inflammation (necrotizing), and
necrotizing without inflammation (scleromalacia perforans). The most
common clinical forms are diffuse scleritis and nodular scleritis. Necrotizing
scleritis with or without inflammation is much less frequent, more ominous,
and frequently associated with systemic autoimmune disorders, as well as
peripheral ulcerative keratitis. Posterior scleritis is characterized by
flattening of the posterior aspect of the globe, thickening of the posterior
coats of the eye (choroid and sclera), and retrobulbar edema.
Pathophysiology

An autoimmune dysregulation in a genetically predisposed host is


presumed to cause scleritis. Inciting factors may include infectious
organisms, endogenous substances, or trauma. The inflammatory process
may be caused by immune complexrelated vascular damage (type III
hypersensitivity) and subsequent chronic granulomatous response (type IV
hypersensitivity).

Epidemiology
Frequency

United States
The exact incidence of scleritis is uncertain, although scleritis is not
common. The reported prevalence of scleritis is skewed by the somewhat
selected referrals of the reporting institutions.

Mortality/Morbidity
Patients with scleritis are at risk for ocular complications and systemic disease
association.

Ocular complications of scleritis, which cause vision loss and eye


destruction, appear as a result of the extending scleral inflammation.
Peripheral ulcerative keratitis (13-14%), uveitis (about 42%), glaucoma (1213%), cataract (6-17%), and fundus abnormalities (about 6.4%). These
complications are most common in necrotizing scleritis, the most
destructive type of scleritis.

Disease association may be found in about 57% of patients with scleritis. Up to 48%
of patients with scleritis present with a known connective-tissue or vasculitic disease.
Some of these diseases are potentially lethal unless treated with prompt and
aggressive therapy. Other patients may present with concomitant trauma, infection,
or postsurgical inflammation. Systemic disease association is most common in cases
of necrotizing scleritis. Scleritis may be the first manifestation of a potentially lethal
systemic disease.
Sex
Women are more likely to have scleritis than men (1.6:1).
Age

Scleritis is most common in the fourth to sixth decades of life. The peak
incidence of scleritis is in the fifth decade.
Prognosis

Prognosis varies depending on the disease classification. It may range


from a self-limited episode of inflammation to a severe necrotizing process
with vision-threatening complication.
Patient Education

It is important for patients with signs and symptoms of scleritis to undergo


an accurate eye evaluation to ensure proper treatment, to determine
prognosis, and to exclude differential diagnoses with less vision-threatening
potential.

Clinical Presentation

History

When interviewing the patient, investigate the following: the major


complaint; a history of the present illness; the past history, including
infection, injury, or surgery; and the review of systems.

Symptoms of scleritis can include pain, tearing or photophobia, tenderness,


and decreased visual acuity. The primary sign is redness.

Pain is the most common symptom for which patients seek medical assistance, and
it is the best indicator of active inflammation. Pain results from both direct stimulation
and stretching of the nerve endings by the inflammation.
The following pain descriptions are characteristic of scleritis:

Severe, penetrating pain that radiates to the forehead, brow, jaw, or sinuses

Awakens the patient during the night

Exacerbated by touch; extremely tender

Only temporarily relieved by analgesics

Tearing or photophobia without mucopurulent discharge, which is usually


mild or moderate, may occur in about 25% of patients with scleritis.

Upon palpation, the patient may describe tenderness that is diffuse with possible
radiation to other parts of the head.

Decreased visual acuity may be caused by extension of scleritis to the adjacent


structures, leading to reactive blepharitis, myositis, keratitis, uveitis, glaucoma,
cataract, and fundus abnormalities.

Redness gradually increases over several days. It has a bluish red tinge, which is
seen best when the examination is performed in natural light, not through the slit

lamp. It may be localized in one sector or involve the whole sclera; most frequently, it
is in the interpalpebral area. This discoloration does not blanche after topical
applications of routine sympathomimetic dilating agents (Neo-Synephrine 2.5%).
Past medical and ocular histories may elucidate systemic diseases, trauma, drugs,
or surgical procedures that might cause scleritis:

Connective-tissue or vasculitic diseases

Infectious diseases

Miscellaneous diseases (eg, atopy, rosacea, gout, chemical injuries)

Blunt or penetrating ocular trauma

Drugs, such as pamidronate (Aredia), alendronate (Fosamax), risedronate


(Actonel), zoledronic acid (Zometa), and ibandronate (Boniva)

Past ocular surgical procedures, especially within a year prior to the onset of
scleritis, might be significant.

Past and present therapies and responses to these interventions should be


investigated.
Because scleritis can be associated with systemic disorders, make a routine inquiry
that covers various bodily systems, as follows:

Dermatologic (eg, skin, hair, nails)

Respiratory

Cardiac

Genitourinary

Rheumatologic

Gastrointestinal

Neurologic

Hematologic and lymphatic

Pulmonary

Ear, nose, sinus, and throat

Physical

The head and extremities (eg, nose, mouth, external ear, skin, joints)
examinations may reveal significant signs, which might be compatible with
a particular underlying disease. An eye examination might detect and
characterize scleral disease. Include scleral and general eye examinations.

Scleral examination
Daylight

The sclera may appear diffuse, deep bluish red, or violaceous. After several attacks
of scleral inflammation, areas of scleral thinning and translucency may appear,
allowing the dark uvea to show.

A black, gray, or brown area that is surrounded by active scleral


inflammation indicates a necrotizing process. If tissue necrosis progresses,
the scleral area may become avascular, producing a white sequestrum in
the center that is surrounded by a well-defined black or dark brown circle.
The slough may be removed gradually by granulation tissue, leaving the
underlying uvea bare or covered by a thin layer of Tenon and conjunctiva.

Slit lamp light


In scleritis, maximum congestion is in the deep episcleral network with
some congestion in the superficial episcleral network. The posterior and
anterior edges of the slit lamp beam are displaced forward because of
underlying scleral and episcleral edema.
In scleritis, topical application of 2.5% or 10% phenylephrine only blanches
the superficial episcleral network without significant effect on the deep
episcleral network.
Red-free light
Red-free light is helpful to the following study areas:

Areas that have maximum vascular congestion


Areas that display new vascular channels
Areas that are totally avascular

General eye examination

Evaluate adjacent structures in scleritis at every follow-up visit, since


involvement is an important reason for vision loss, as follows:

Extraocular muscles
Lids and orbit
Cornea
Uvea
Lens
Intraocular pressure (IOP)


Dilated fundus
Causes
Scleritis may occur isolated (43%) or in association with several types of
disorders (57%), as follows:

Autoimmune (48%)
o
Connective-tissue diseases and other inflammatory conditions
include the following [3] :

Rheumatoid arthritis

Systemic lupus erythematosus

Ankylosing spondylitis

Reactive arthritis

Psoriatic arthritis

Gouty arthritis

Inflammatory bowel diseases

Relapsing polychondritis

Polymyositis

Sjgren syndrome

Mixed connective tissue disease

Progressive systemic sclerosis


o
Vasculitic diseases include the following [4] :

Polyarteritis nodosa

Allergic angiitis of Churg-Strauss syndrome

Granulomatosis with polyangiitis (Wegener


granulomatosis)

Behet disease

Giant cell arteritis

Cogan syndrome

Infectious (7%) - Bacterial, fungal, viral, or parasitic

Miscellaneous (2%) - Atopy; rosacea; or secondary to foreign bodies,


chemical injuries, or drugs (eg, pamidronate, alendronate, risedronate,
zoledronic acid, ibandronate)
Workup

Laboratory Studies

Based on the past history, review of systems, and physical examination,


select appropriate diagnostic tests to confirm or reject the following
suspected associated diseases:

Rheumatoid factor - Rheumatoid arthritis


Antinuclear antibodies - Systemic lupus erythematosus, rheumatoid
arthritis, polymyositis, progressive systemic sclerosis, or mixed
connective tissue
Antineutrophil cytoplasmic antibodies (ANCA) - Granulomatosis with
polyangiitis, polyarteritis nodosa, or microscopic polyangiitis
Human leukocyte antigen (HLA) typing - Ankylosing spondylitis,
reactive arthritis, psoriatic arthritis, or arthritis associated with
inflammatory bowel disease
Eosinophil count/immunoglobulin E (IgE) - Allergic angiitis of ChurgStrauss syndrome or atopy
Uric acid - Gout
Erythrocyte sedimentation rate (ESR) - Giant cell arteritis
Hepatitis B surface antigen (HBsAg) - Polyarteritis nodosa
Serologies - Infectious diseases, including syphilis and Lyme disease
Purified-protein derivative (PPD) skin test or Quantiferon gold assay Tuberculosis
Anergy skin test - Sarcoidosis
Prick test - Atopy

Imaging Studies

See the list below:

Chest radiography or CT scanning - Tuberculosis, Granulomatosis


with polyangiitis, allergic angiitis of Churg-Strauss syndrome,
sarcoidosis, or atopy
Sinus films - Granulomatosis with polyangiitis
Sacroiliac radiography - Ankylosing spondylitis, reactive arthritis,
psoriatic arthritis, or arthritis associated with inflammatory bowel
disease
Limb joint radiography - Rheumatoid arthritis, psoriatic arthritis,
arthritis associated with inflammatory bowel disease, or gout
Ultrasonography (A-scan and B-scan) - Posterior scleritis; most
helpful test to aid in diagnosing posterior scleritis, which is
characterized by flattening of the posterior aspect of the globe,
thickening of the posterior sclera, and retrobulbar edema [6]
Orbital CT scanning - Posterior scleritis; a useful diagnostic tool to
aid in detecting the following, which are important to differentiate
posterior scleritis from orbital inflammatory diseases and orbital
neoplasm: extraocular muscle or lacrimal gland enlargement, sinus
tissue involvement, and posterior scleral thickening [7]

Orbital MRI - Posterior scleritis


o
MRI is used to differentiate localized inflammatory
pseudotumor from posterior scleritis in proptosis or choroidal
tumors from posterior scleritis in subretinal mass.
o
Some orbital tumors can cause choroidal folds and retinal
striae, which are also signs of posterior scleritis, and are detected
reliably with MRI.

Related clinical guideline summary from the American College of


Radiology:ACR Appropriateness Criteria: Orbits, vision, and visual
loss.
Other Tests
See the list below:

Smears, cultures, and polymerase chain reaction (PCR) from


conjunctival, corneal, episcleral, or scleral scraping - Infectious scleritis
o
Scleral or corneoscleral biopsy is recommended if smears and
culture results are negative at 48 hours, infectious scleritis is still
the primary clinical suspicion, and the patient is worsening despite
targeted empirical antimicrobial therapy.

One third of tissue from a biopsy is sent to the


microbiology department, where it is homogenized for
smears, cultures, and PCR.

The middle third of tissue is transported to the pathology


department for histopathology with special stains (eg, periodic
acid-Schiff [PAS], Gomori methenamine-silver [GMS], acidfast, calcofluor white).

The last third of tissue is sent to the immunology


department for immunofluorescence studies with monoclonal
antibodies (eg, antiherpes simplex virus type 1, anti
varicella-zoster virus antibodies).

PCR of body fluids, orbital abscess drainage, aqueous humor, and


vitreous - Infectious scleritis
Histologic Findings
Diffuse scleritis or nodular scleritis

A nongranulomatous inflammatory reaction occurs that is characterized by


infiltration of mononuclear cells, such as macrophages, lymphocytes, and
plasma cells. In the most severe cases, mononuclear cells may organize
into granulomatous lesions. Mast cells, neutrophils, and eosinophils may
also be present.

Necrotizing scleritis

A granulomatous inflammatory reaction occurs that is characterized by a


central area of fibrinoid necrosis, surrounded by epithelioid cells,
multinucleated giant cells, lymphocytes, and plasma cells. Neutrophils,
mast cells, and eosinophils are dispersed throughout the inflamed tissue
and around vessels. Inflammatory microangiopathy, which is characterized
by neutrophilic infiltration in and around the episclera and sclera that
perforates the vessel walls with or without fibrinoid necrosis, is frequently
seen.

Treatment & Management

Medical Care

Treatment of scleritis almost always requires systemic therapy. Patients


with an associated disease, such as rosacea, gout, atopy, or infection,
need disease-specific treatment. Systemic therapy complements
aggressive topical corticosteroid therapy, generally with difluprednate,
prednisolone or loteprednol etabonate, thereby reducing the dose
requirements for both topical and oral modalities. This combination therapy
mitigates, to some extent, the inherent risks of oral steroids, including
hypertension and diabetes, among many others; the risks of GI and renal
morbidity caused by oral nonsteroidal anti-inflammatory drugs (NSAIDs);
and the risks of elevated IOP and cataractogenesis due to topical steroids.

Treatment of noninfectious scleritis: Systemic NSAIDs, corticosteroids, or


immunomodulatory drugs are indicated. [10] Topical therapy is routinely insufficient.
This treatment must be individualized for the severity of the scleritis, response to
treatment, adverse effects, and presence of associated disease.
Dosing information is as follows:

Methotrexate 7.5-25 mg PO/IM once a week

Azathioprine 1-2.5 mg/kg/day PO

Cyclophosphamide 1.5-3 mg/kg/day PO

Mycophenolate mofetil 1 g PO bid

Infliximab 3-5 mg/kg IV q6-8wk

Diffuse scleritis or nodular scleritis


The initial therapy consists of an NSAID; in case of therapeutic failure, 2 additional
successive different NSAIDs should be tried following the first drug. In high-risk
patients, consider appropriate gastrointestinal protection with misoprostol or
omeprazole.
If NSAIDs are not effective or have untoward complications, oral corticosteroids can
be substituted. Remission may be maintained with continued NSAIDs.
Periorbital and subconjunctival steroid injections have been reported to be
efficacious as adjunctive therapy. Caution should be observed, particularly when a
comorbid infectious etiology such as toxoplasmosis or syphilis cannot be completely
ruled out.

In case of therapeutic failure of systemic corticosteroids, immunosuppressive drugs


should be added or substituted. Methotrexate (MTX) can be the first choice, but
azathioprine, mycophenolate mofetil, cyclophosphamide, or cyclosporine may also
be helpful. [12, 13, 14] Cyclophosphamide should be the first choice in treating patients
with associated potentially lethal vasculitic diseases, such as granulomatosis with
polyangiitis or polyarteritis nodosa.

In case of therapeutic failure, biologic response modifiers, such as infliximab or


adalimumab, may be effective. [15, 16, 17, 1, 6] Other alternatives include golimumab,
certolizumab, tocilizumab, and rituximab, although further investigation is warranted.

Necrotizing scleritis
The initial therapy consists of immunosuppressive drugs that are supplemented with
corticosteroids during the first month; the latter is tapered slowly, if possible.
Cyclophosphamide should be the first choice in treating patients with an underlying
systemic vasculitis such as granulomatosis with polyangiitis or polyarteritis nodosa.
In case of therapeutic failure, biologic response modifiers, such as infliximab or
adalimumab, may be effective. Other alternatives are golimumab, certolizumab,
tocilizumab, and rituximab, although their efficacy awaits further study. [20, 21]
Periocular steroid injections should not be applied in cases of necrotizing scleritis or
peripheral ulcerative keratitis but could be very helpful in diffuse or nodular scleritis
as an adjunctive therapy. Some authors believe that depot steroids actually may
exacerbate necrotizing disease.

Pulse intravenous cyclophosphamide with or without pulse intravenous


corticosteroids may be required for urgent cases and may be followed by
maintenance therapy.
Infectious scleritis
Systemic treatment with or without topical antimicrobial therapy always is required.
Differentiating infectious scleritis from noninfectious scleritis is important because
corticosteroid therapy and immunosuppressive therapy (often used in noninfectious
autoimmune scleritis) are contraindicated in active infections.

Surgical Care
Tectonic surgical procedures rarely may be required to preserve the integrity of the
globe.
Scleral grafts from fresh donor sclera or glycerin-preserved sclera are available
through eye banks. Grafts may be performed in cases of pending perforation during
the time before the effects of systemic immunosuppressive agents manifest.
Corneal tissue may be used for associated corneal disease.

Consultations
See the list below:

Consultation with a rheumatologist or internal medicine specialist for associated


disease
Consultation with a hematologist, oncologist, or internal medicine specialist for
immunomodulatory therapy

Precautions

Drug precautions are as follows:

Avoid methotrexate if creatinine clearance <10; contraindicated in


chronic hepatic disease;, screen for hepatitis A, B, and C; give
hepatitis vaccination; decrease or avoid alcohol intake

Azathioprine is associated with an increased risk of pancreatitis;


decrease dose 50% if creatinine clearance <10

Decrease cyclophosphamide dose 25% if creatinine clearance <10;


caution advised in patients with hepatic impairment

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent


complications.
Corticosteroids
Class Summary

Have anti-inflammatory properties and cause profound and varied


metabolic effects. Corticosteroids modify the body's immune response to
diverse stimuli.
Prednisone (Deltasone, Rayos)

View full drug information

Inhibits phospholipase A and Fc receptor expression, activates the


glucocorticoid receptor, reduces cytokine production, suppresses
lymphocyte function, and redistributes circulating leukocytes. Also potent
inhibitors of angiogenesis and potent stabilizer of cell membranes.
Prednisolone (FloPred, Millipred, Millipred DP, Prelone, Veripred 20)

View full drug information

Corticosteroids act as potent inhibitors of inflammation. They may cause


profound and varied metabolic effects, particularly in relation to salt, water,
and glucose tolerance, in addition to their modification of the immune
response of the body. Alternative corticosteroids may be used in equivalent
dosage.
Immunosuppressive drugs
Class Summary

Inhibit specific immune system pathways.


Methotrexate (Trexall Rheumatrex, Otrexup, Rasuvo)

View full drug information

Inhibits DHFR, causing a block in the reduction of dihydrofolate to


tetrahydrofolate. This inhibits the formation of thymidylate and purines;
arrests DNA, RNA, and protein synthesis. Patients should take adjunctive

folic acid therapy up to 1000 mg per day to avoid hematopoietic


complications.
Azathioprine (Imuran, Azasan)

View full drug information

Interferes with DNA synthesis and inhibits lymphocyte proliferation.


Cyclophosphamide

View full drug information

Chemically related to nitrogen mustards. As an alkylating agent, the


mechanism of action of the active metabolites may involve cross linking of
DNA, which may interfere with growth of normal and neoplastic cells. Drug
of choice for granulomatosis with polyangiitis or polyarteritis nodosa.
Cyclosporine (Neoral, Sandimmune, Gengraf)

View full drug information

Cyclic polypeptide that suppresses some humoral immunity and, to a


greater extent, cell-mediated immune reactions, such as delayed
hypersensitivity. For children and adults, base dosing on ideal body weight.
Mycophenolate mofetil (CellCept, Myfortic)

View full drug information

Inhibits purine synthesis and proliferation of human lymphocytes. Promising


published case report of 3 patients with resistant disease treated with
mycophenolate mofetil. Reduced toxicity makes this regimen an attractive
alternative.
Monoclonal antibodies
Class Summary

Selective immunomodulators that affect specific aspects of the


inflammatory pathways.
Adalimumab (Humira)

View full drug information

Subcutaneous recombinant human IgG1 monoclonal antibody specific for


human TNF. Indicated to reduce inflammation and inhibit progression of
structural damage in moderate-to-severe rheumatoid arthritis. Reserved for
those who experience inadequate response to one or more DMARDs. Can
be used alone or in combination with MTX or other DMARDs. Binds
specifically to TNF-alpha and blocks interaction with p55 and p75 cellsurface TNF receptors.
Infliximab (Remicade)

View full drug information

Intravenous chimeric IgG1k monoclonal antibody that neutralizes cytokine


TNF-alpha and inhibits its binding to TNF-alpha receptor. Reduces
infiltration of inflammatory cells and TNF-alpha production in inflamed
areas. Used with MTX in patients who have had inadequate response to
MTX monotherapy.
Rituximab (Rituxan)

View full drug information

Indicated to reduce signs and symptoms for moderately-to-severely active


rheumatoid arthritis in combination with MTX. For use in adults who have
experienced an inadequate response to one or more TNF antagonist
therapies. Antibody genetically engineered. Chimeric murine/human
monoclonal antibody directed against the CD20 antigen found on surface of
B lymphocytes. Focal leukoencephalopathy is a lethal rare side effect that
must be considered.
Nonsteroidal anti-inflammatory drugs
Class Summary

Have analgesic, anti-inflammatory, and antipyretic activities. Their


mechanism of action is not known but may inhibit cyclooxygenase activity
and prostaglandin synthesis. Other mechanisms may exist, such as
inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase
activity, neutrophil aggregation, and various cell membrane functions.
Systemic therapy with NSAIDs, corticosteroids, and immunosuppressive
agents, alone or in combination, may be effective in patients with
noninfectious scleritis.

Ibuprofen (Motrin, Provil, Addaprin, Caldolor, Advil, Dyspel)

View full drug information

DOC for patients with mild to moderate pain. Inhibits inflammatory


reactions and pain by decreasing prostaglandin synthesis.
Indomethacin (Indocin, Tivorbex)

View full drug information

Rapidly absorbed. Metabolism occurs in liver by demethylation,


deacetylation, and glucuronide conjugation. Inhibits prostaglandin
synthesis.
Naproxen (Naprosyn, Naprelan, Aleve, Anaprox DS)

View full drug information

For relief of mild to moderate pain; inhibits inflammatory reactions and pain
by decreasing activity of cyclooxygenase, which results in a decrease of
prostaglandin synthesis.
Piroxicam (Feldene)

View full drug information

Decreases activity of cyclooxygenase, which, in turn, inhibits prostaglandin


synthesis. These effects decrease formation of inflammatory mediators.
Celecoxib (Celebrex)

View full drug information

Primarily inhibits COX-2. COX-2 is considered an inducible isoenzyme,


induced by pain and inflammatory stimuli. Inhibition of COX-1 may
contribute to NSAID GI toxicity. At therapeutic concentrations, COX-1
isoenzyme is not inhibited, thus incidence of GI toxicity, such as
endoscopic peptic ulcers, bleeding ulcers, perforations, and obstructions,
may be decreased when compared to nonselective NSAIDs. Seek lowest
dose for each patient.
Neutralizes circulating myelin antibodies through anti-idiotypic antibodies;
down-regulates proinflammatory cytokines, including INF-gamma; blocks
Fc receptors on macrophages; suppresses inducer T and B cells and

augments suppressor T cells; blocks complement cascade; promotes


remyelination; may increase CSF IgG (10%).
Has a sulfonamide chain and is primarily dependent upon cytochrome
P450 enzymes (a hepatic enzyme) for metabolism.

Further Outpatient Care

Patients should undergo a room-light scleral examination, a slit-lamp


evaluation, and general eye examination at every follow-up visit.
Carefully monitor medication type, dose, and adverse effects. In case of
therapeutic failure, change the medication type.
Consultation with a rheumatologist for associated systemic disease and/or
immunomodulatory therapy follow-up care is recommended.
Further Inpatient Care

In cases of pending scleral perforation, peripheral ulcerative keratitis


perforation, or positive Seidel test, admit for scleral patch grafting.
Inpatient & Outpatient Medications

Systemic medications include nonsteroidal anti-inflammatory drugs


(NSAIDs), corticosteroids, or immunomodulatory agents, depending on the
type of scleritis and/or associated disease.
Complications

See the list below:

Keratopathy - Peripheral corneal thinning, acute stromal keratitis,


sclerosing keratitis, interstitial keratitis, punctate keratopathy, or
peripheral ulcerative keratitis
Uveitis - Anterior or posterior
Glaucoma
Cataract
Fundus abnormalities - Choroidal folds, subretinal mass, disk edema,
macular edema, annular ciliochoroidal detachment, or serous retinal
detachment

Prognosis

Ocular prognosis of scleritis with systemic autoimmune diseases varies,


depending on the specific autoimmune disease.
Scleritis in spondyloarthropathies or in systemic lupus erythematosus,
usually a relatively benign and self-limiting condition, is diffuse scleritis or
nodular scleritis without ocular complications.
Scleritis in granulomatosis with polyangiitis is a severe disease that can
lead to permanent blindness; it is usually necrotizing scleritis with ocular
complications.
Scleritis in rheumatoid arthritis or relapsing polychondritis is a disease of
intermediate severity; it may be diffuse, nodular, or necrotizing scleritis with
or without ocular complications.
Scleritis without systemic disease association is often more benign than
scleritis accompanied by infection or autoimmune disease. These cases of
idiopathic scleritis may be mild, shorter in duration, and more likely to
respond to topical steroid drops alone.