Scleritis

Inflammation of entire thickness of the sclera
Specialty| Ophthalmology, Optometry
Symptoms| Eye redness, pain, photophobia, tearing, decrease in visual acuity
Usual onset| 30 - 60 years of age
Risk factors| Female gender
Differential diagnosis| Episcleritis
Treatment| Non-steroidal anti-inflammatory drugs
Surgery (rarely needed)
Frequency| Uncommon

Scleritis is a serious inflammatory disease that affects the white outer coating of the eye, known as the sclera. The disease is often contracted through association with other diseases of the body, such as granulomatosis with polyangiitis or rheumatoid arthritis. There are three types of scleritis: diffuse scleritis (the most common), nodular scleritis, and necrotizing scleritis (the most severe). Scleritis may be the first symptom of onset of connective tissue disease.[1]

Episcleritis is inflammation of the episclera, a less serious condition that seldom develops into scleritis.[2]

## Contents

* 1 Signs and symptoms
* 1.1 Complications
* 2 Pathophysiology
* 3 Diagnosis
* 3.1 Classification
* 4 Treatment
* 4.1 Medical
* 4.2 Surgical Intervention
* 5 Epidemiology
* 6 References
* 7 External links

## Signs and symptoms

Scleral translucency following recurrent scleritis.

Symptoms of scleritis include:[3]

* Redness of the sclera and conjunctiva, sometimes changing to a purple hue
* Severe ocular pain, which may radiate to the temple or jaw. The pain is often described as deep or boring.
* Photophobia and tearing
* Decrease in visual acuity, possibly leading to blindness

The pain of episcleritis is less severe than in scleritis.[4] In hyperemia, there is a visible increase in the blood flow to the sclera (hyperaemia), which accounts for the redness of the eye. Unlike in conjunctivitis, this redness will not move with gentle pressure to the conjunctiva.

### Complications

Secondary keratitis or uveitis may occur with scleritis.[4] The most severe complications are associated with necrotizing scleritis.

## Pathophysiology

Most of the time, scleritis is not caused by an infectious agent.[5] Histopathological changes are that of a chronic granulomatous disorder, characterized by fibrinoid necrosis, infiltration by polymorphonuclear cells, lymphocytes, plasma cells and macrophages. The granuloma is surrounded by multinucleated epitheloid giant cells and new vessels, some of which may show evidence of vasculitis.

## Diagnosis

Scleritis is best detected by examining the sclera in daylight; retracting the lids helps determine the extent of involvement. Other aspects of the eye exam (i.e. visual acuity testing, slit lamp examination, etc.) may be normal. Scleritis may be differentiated from episcleritis by using phenylephrine eye drops, which causes blanching of the blood vessels in episcleritis, but not in scleritis.[4]

Ancillary tests CT scans, MRIs, and ultrasonographies can be helpful, but do not replace the physical examination.

### Classification

Scleritis can be classified as anterior scleritis and posterior scleritis. Anterior scleritis is the most common variety, accounting for about 98% of the cases. It is of two types : Non-necrotising and necrotising. Non-necrotising scleritis is the most common, and is further classified into diffuse and nodular type based on morphology. Necrotising scleritis accounts for 13% of the cases. It can occur with or without inflammation.

## Treatment

### Medical

In mild to moderate cases of scleritis, non-steroidal anti-inflammatory drugs such as flurbiprofen, indomethacin or ibuprofen may be prescribed for pain relief.[6] Systemic corticosteroids like prednisolone may be used if NSAIDs are inappropriate or scleritis is posterior or necrotizing.[7] Periocular steroid injections may be used in non-necrotizing scleritis, but it is contraindicated in necrotizing disease.[7] If disease control is inadequate with steroids alone, immunosuppressives (Eg: cyclophosphamide, azathioprine, methotrexate) and/or immunomodulators may be considered for treatment.[7] In infective scleritis, if infective agent is identified, topical or systemic antibiotics may be prescribed.[7][8]

### Surgical Intervention

Surgery may be indicated if scleral perforation or excessive scleral thinning is present.[6] Bandage contact lens or corneal glue may be used to repair damaged corneal tissue in the eye and preserve the patient's vision.[6] If not treated, scleritis can cause blindness.

## Epidemiology

Scleritis is not a common disease, although the exact prevalence and incidence are unknown. It is somewhat more common in women, and is most common in the fourth to sixth decades of life.[9]

## References

1. ↑ "Scleritis". WebMD, LLC. Medscape. http://emedicine.medscape.com/article/1228324-overview.
2. ↑ "Episcleritis: MedlinePlus Medical Encyclopedia". Bethesda, MD: United States National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001019.htm.
3. ↑ Vorvick, Linda J. (July 28, 2010). "Scleritis". PubMed Health. United States National Library of Medicine. https://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001998/.
4. ↑ 4.0 4.1 4.2 Goldman, Lee (2011). Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. pp. 2440. ISBN 978-1437727883.
5. ↑ Yanoff, Myron; Jay S. Duker (2008). Ophthalmology (3rd ed.). Edinburgh: Mosby. pp. 255–261. ISBN 978-0323057516.
6. ↑ 6.0 6.1 6.2 "Scleritis". https://eyewiki.aao.org/Scleritis.
7. ↑ 7.0 7.1 7.2 7.3 John F., Salmon (2020). "Episclera and sclera". Kanski's clinical ophthalmology : a systematic approach (9th ed.). Edinburgh: Elsevier. ISBN 978-0-7020-7713-5. OCLC 1131846767. https://www.worldcat.org/oclc/1131846767.
8. ↑ Ramanjit, Sihota; Radhika, Tandon (2015). Parsons' diseases of the eye (22nd ed.). New Delhi, India: Elsevier. ISBN 978-81-312-3819-6. OCLC 905915528.
9. ↑ Maite Sainz de la Maza (Feb 15, 2012). The sclera (2nd ed.). New York: Springer. pp. 102. ISBN 978-1441965011.

* Rosenbaum JT. The Eye and rheumatic diseases. In: Firestein GS, Budd RC, Harris ED Jr, et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008:chap 46.
* Watson P. Diseases of the sclera and episclera. In: Tasman W, Jaeger EA, eds. Duane’s Ophthalmology. 15th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2009:chap 23.

## External links

Classification|

* ICD-10: H15.0
* ICD-9-CM: 379.0
* MeSH: D015423
* DiseasesDB: 11898

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External resources|

* MedlinePlus: 001003
* eMedicine: emerg/521 oph/642

* v
* t
* e

* Diseases of the human eye (H00–H59
* 360–379)

Adnexa

| Eyelid|

| Inflammation|

* Stye
* Chalazion
* Blepharitis

|

* Entropion
* Ectropion
* Lagophthalmos
* Blepharochalasis
* Ptosis
* Blepharophimosis
* Xanthelasma

Eyelash|

* Trichiasis
* Madarosis

Lacrimal apparatus|

* Dacryoadenitis
* Epiphora
* Dacryocystitis
* Xerophthalmia

Orbit|

* Exophthalmos
* Enophthalmos
* Orbital cellulitis
* Orbital lymphoma
* Periorbital cellulitis

Conjunctiva|

* Conjunctivitis
* allergic
* Pterygium
* Pinguecula
* Subconjunctival hemorrhage

Globe

| Fibrous tunic|

| Sclera|

* Scleritis
* Episcleritis

|

Cornea|

* Keratitis
* herpetic
* acanthamoebic
* fungal
* Corneal ulcer
* Photokeratitis
* Thygeson's superficial punctate keratopathy
* Corneal dystrophy
* Fuchs'
* Meesmann
* Corneal ectasia
* Keratoconus
* Pellucid marginal degeneration
* Keratoglobus
* Terrien's marginal degeneration
* Post-LASIK ectasia
* Keratoconjunctivitis
* sicca
* Corneal neovascularization
* Kayser–Fleischer ring
* Haab's striae
* Arcus senilis
* Band keratopathy

Vascular tunic|

* Iris
* Ciliary body

* Uveitis
* Intermediate uveitis
* Hyphema
* Rubeosis iridis
* Persistent pupillary membrane
* Iridodialysis
* Synechia

|

Choroid|

* Choroideremia
* Choroiditis
* Chorioretinitis

Lens|

* Cataract
* Congenital cataract
* Childhood cataract
* Aphakia
* Ectopia lentis

Retina|

* Retinitis
* Chorioretinitis
* Cytomegalovirus retinitis
* Retinal detachment
* Retinoschisis
* Ocular ischemic syndrome / Central retinal vein occlusion
* Central retinal artery occlusion
* Branch retinal artery occlusion
* Retinopathy
* diabetic
* hypertensive
* Purtscher's
* of prematurity
* Bietti's crystalline dystrophy
* Coats' disease
* Macular degeneration
* Retinitis pigmentosa
* Retinal haemorrhage
* Central serous retinopathy
* Macular edema
* Epiretinal membrane (Macular pucker)
* Vitelliform macular dystrophy
* Leber's congenital amaurosis
* Birdshot chorioretinopathy

Other|

* Glaucoma / Ocular hypertension / Primary juvenile glaucoma
* Floater
* Leber's hereditary optic neuropathy
* Red eye
* Globe rupture
* Keratomycosis
* Phthisis bulbi
* Persistent fetal vasculature / Persistent hyperplastic primary vitreous
* Persistent tunica vasculosa lentis
* Familial exudative vitreoretinopathy

Pathways

| Optic nerve
Optic disc|

* Optic neuritis
* optic papillitis
* Papilledema
* Foster Kennedy syndrome
* Optic atrophy
* Optic disc drusen

Optic neuropathy|

* Ischemic
* anterior (AION)
* posterior (PION)
* Kjer's
* Leber's hereditary
* Toxic and nutritional

Strabismus
Extraocular muscles
Binocular vision
Accommodation|

| Paralytic strabismus|

* Ophthalmoparesis
* Chronic progressive external ophthalmoplegia
* Kearns–Sayre syndrome

palsies|

* Oculomotor (III)
* Fourth-nerve (IV)
* Sixth-nerve (VI)

Other strabismus|

* Esotropia / Exotropia
* Hypertropia
* Heterophoria
* Esophoria
* Exophoria
* Cyclotropia
* Brown's syndrome
* Duane syndrome

Other binocular|

* Conjugate gaze palsy
* Convergence insufficiency
* Internuclear ophthalmoplegia
* One and a half syndrome

Refraction|

* Refractive error
* Hyperopia
* Myopia
* Astigmatism
* Anisometropia / Aniseikonia
* Presbyopia

Vision disorders
Blindness|

* Amblyopia
* Leber's congenital amaurosis
* Diplopia
* Scotoma
* Color blindness
* Achromatopsia
* Dichromacy
* Monochromacy
* Nyctalopia
* Oguchi disease
* Blindness / Vision loss / Visual impairment

Anopsia|

* Hemianopsia
* binasal
* bitemporal
* homonymous
* Quadrantanopia

subjective|

* Asthenopia
* Hemeralopia
* Photophobia
* Scintillating scotoma

Pupil|

* Anisocoria
* Argyll Robertson pupil
* Marcus Gunn pupil
* Adie syndrome
* Miosis
* Mydriasis
* Cycloplegia
* Parinaud's syndrome

Other|

* Nystagmus
* Childhood blindness

Infections

* Trachoma
* Onchocerciasis

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