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Conjunctivitis is part of the triad of reactive arthritis, which is thought to be caused by autoimmune cross-reactivity following certain bacterial infections. Reactive arthritis is highly associated with HLA-B27. Conjunctivitis is associated with the autoimmune disease relapsing polychondritis.
Conjunctivitis may also be caused by allergens such as pollen, perfumes, cosmetics, smoke, dust mites, Balsam of Peru, and eye drops.
Episcleritis is a common disease, and its exact prevalence and incidence are unknown. It typically affects young adults, and may be more common in women.
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.
Episcleritis is a benign, self-limiting condition, meaning patients recover without any treatment. Most cases of episcleritis resolve within 7–10 days. The nodular type is more aggressive and takes longer to resolve. Although rare, some cases may progress to scleritis. However, in general, episcleritis does not cause complications in the eye. Smoking tobacco delays the response to treatment in patients with episcleritis.
Most of the time, scleritis is not caused by an infectious agent. 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.
By far the most common cause of IK is syphilitic disease. However, there are two possible causes of the corneal inflammatory response: an infection and/or an immunological response, such as a hypersensitivity type reaction, or (rarely) Cogan syndrome. Infectious causes include syphilis (commonest), followed by other bacterial infections (TB, Leprosy and Lyme disease) and parasitic infections (Acanthamoeba, Onchocerciasis or river blindness, Leishmaniasis, Trypanosoma cruzi or "Chagas disease", Trypanosoma brucei or "African sleeping sickness" and microsporidia)
Interstitial keratitis (IK) is corneal scarring due to chronic inflammation of the corneal stroma. Interstitial means space between cells i.e. corneal stroma which lies between the epithelium and the endothelium. Keratitis means corneal inflammation.
Late congenital syphilitic oculopathy is a disease of the eye, a manifestation of late congenital syphilis. It can appear as:
- Interstitial keratitis – this commonly appears between ages 6 and 12. Symptoms include lacrimation and photophobia. Pathological vascularization of the cornea cause it to turn pink or salmon colored. 90% of cases affect both eyes.
- Episcleritis or scleritis – nodules appear in or overlying the sclera (white of eye)
- Iritis or iris papules – vascular infiltration of the iris causes rosy color change and yellow/red nodules.
- Chorioretinitis, papillitis, retinal vasculitis – retinal changes can resemble retinitis pigmentosa.
- Exudative retinal detachment
Congenital syphilis is categorized by the age of the child. Early congenital syphilis occurs in children under 2 years old, and late congenital syphilis in children at or greater than 2 years old. Manifestations of late congenital syphilis are similar to those of secondary syphilis and tertiary syphilis in adults.
Herpes zoster ophthalmicus (HZO) and also known as ophthalmic zoster is a disease characterised by reactivation of dormant varicella zoster virus residing within the ophthalmic nerve (the first division of the trigeminal nerve). This condition is an important subtype of shingles, representing 15% of all cases.
Herpes zoster ophthalmicus is transmitted via direct contact or droplets. Varicella zoster virus is a DNA virus which produces acidophilic intranuclear inclusion bodies. The virus is neurotrophic in nature.
The frontal nerve is more commonly affected than the nasociliary nerve or lacrimal nerve.
Any potential ocular involvement should be assessed by an ophthalmologist as complications such as episcleritis and uveitis may occur.
Of the many causes, conjunctivitis is the most common. Others include:
"Usually nonurgent"
- blepharitis - a usually chronic inflammation of the eyelids with scaling, sometimes resolving spontaneously
- subconjunctival hemorrhage - a sometimes dramatic, but usually harmless, bleeding underneath the conjunctiva most often from spontaneous rupture of the small, fragile blood vessels, commonly from a cough or sneeze
- inflamed pterygium - a benign, triangular, horizontal growth of the conjunctiva, arising from the inner side, at the level of contact of the upper and lower eyelids, associated with exposure to sunlight, low humidity and dust. It may be more common in occupations such as farming and welding.
- inflamed pinguecula - a yellow-white deposit close to the junction between the cornea and sclera, on the conjunctiva. It is most prevalent in tropical climates with much UV exposure. Although harmless, it can occasionally become inflamed.
- dry eye syndrome - caused by either decreased tear production or increased tear film evaporation which may lead to irritation and redness
- airborne contaminants or irritants
- tiredness
- drug use including cannabis
"Usually urgent"
- acute angle closure glaucoma - implies injury to the optic nerve with the potential for irreversible vision loss which may be permanent unless treated quickly, as a result of increased pressure within the eyeball. Not all forms of glaucoma are acute, and not all are associated with increased 'intra-ocular' pressure.
- injury
- keratitis - a potentially serious inflammation or injury to the cornea (window), often associated with significant pain, light intolerance, and deterioration in vision. Numerous causes include virus infection. Injury from contact lenses can lead to keratitis.
- iritis - together with the ciliary body and choroid, the iris makes up the uvea, part of the middle, pigmented, structures of the eye. Inflammation of this layer (uveitis) requires urgent control and is estimated to be responsible for 10% of blindness in the United States.
- scleritis - a serious inflammatory condition, often painful, that can result in permanent vision loss, and without an identifiable cause in half of those presenting with it. About 30-40% have an underlying systemic autoimmune condition.
- episcleritis - most often a mild, inflammatory disorder of the 'white' of the eye unassociated with eye complications in contrast to scleritis, and responding to topical medications such as anti-inflammatory drops.
- tick borne illnesses like Rocky Mountain spotted fever - the eye is not primarily involved, but the presence of conjunctivitis, along with fever and rash, may help with the diagnosis in appropriate circumstances.
A red eye is an eye that appears red due to illness or injury. It is usually injection and prominence of the superficial blood vessels of the conjunctiva, or sclera, which may be caused by disorders of these or adjacent structures. Conjunctivitis and subconjunctival hemorrhage are two of the less serious but more common causes.
Management includes assessing whether emergency action (including referral) is needed, or whether treatment can be accomplished without additional resources.
Slit lamp examination is invaluable in diagnosis but initial assessment can be performed using a careful history, testing vision (visual acuity), and carrying out a penlight examination.
This is a partial list of human eye diseases and disorders.
The World Health Organization publishes a classification of known diseases and injuries, the International Statistical Classification of Diseases and Related Health Problems, or ICD-10. This list uses that classification.
The following are not classified as diseases of the eye and adnexa (H00-H59) by the World Health Organization:
- (B36.1) Keratomycosis — fungal infection of the cornea
- (E50.6-E50.7) Xerophthalmia — dry eyes, caused by vitamin A deficiency
- (Q13.1) Aniridia — a rare congenital eye condition leading to underdevelopment or even absence of the iris of the eye
Causes of photophobia relating directly to the eye itself include:
- Achromatopsia
- Aniridia
- Anticholinergic drugs may cause photophobia by paralyzing the iris sphincter muscle.
- Aphakia (absence of the lens of the eye)
- Blepharitis
- Buphthalmos (abnormally narrow angle between the cornea and iris)
- Cataracts
- Coloboma
- Cone dystrophy
- Congenital abnormalities of the eye
- Viral conjunctivitis ("pink eye")
- Corneal abrasion
- Corneal dystrophy
- Corneal ulcer
- Disruption of the corneal epithelium, such as that caused by a corneal foreign body or keratitis
- Ectopia lentis
- Endophthalmitis
- Eye trauma caused by disease, injury, or infection such as chalazion, episcleritis, glaucoma, keratoconus, or optic nerve hypoplasia
- Hydrophthalmos, or congenital glaucoma
- Iritis
- The drug isotretinoin (Accutane/Roaccutane) has been associated with photophobia
- Optic neuritis
- Pigment dispersion syndrome
- Pupillary dilation (naturally or chemically induced)
- Retinal detachment
- Scarring of the cornea or sclera
- Uveitis
Many individuals have mild symptoms, which recur infrequently, while others may have persistent problems that become debilitating or life-threatening.
Neurological causes for photophobia include:
- Autism spectrum disorders
- Chiari malformation
- Occipital Neuralgia
- Dyslexia
- Encephalitis including Myalgic encephalomyelitis aka Chronic fatigue syndrome
- Meningitis
- Trigeminal disturbance causes central sensitization (hence, multiple other associated hypersensitivities. Causes can be bad bite, infected tooth, etc.
- Subarachnoid haemorrhage
- Tumor of the posterior cranial fossa
A limbal nodule is any nodular lesion at the limbus (junction of the cornea and sclera) of the eye.
The differential diagnosis for a limbal nodule can include:
- Pinguecula
- Early Pterygium
- Foreign body / foreign body granuloma
- Phlycten, an inflamed nodule of lymphoid tissue
- Episcleritis
- Scleritis
- Granuloma
- Limbal dermoid, a kind of choristoma (NB: in other organs "dermoid" can refer to a teratoma)
- malignant melanoma
Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage.
Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) are their own are separate entities.
Relapsing polychondritis is an autoimmune disease in which the body's immune system begins to attack and destroy the cartilage tissues in the body. It has been postulated that both cell-mediated immunity and humoral immunity are responsible.
Reasons for disease onset are not known, but there is no evidence of a genetic predisposition to developing relapsing polychondritis. However, there are cases where multiple members of the same family have been diagnosed with this illness. Studies indicate that some genetic contribution to susceptibility is likely.
Before modern treatments, the 2-year mortality was over 90% and average survival five months. Death usually resulted from uremia or respiratory failure.
With corticosteroids and cyclophosphamide, 5-year survival is over 80%. Long-term complications are common (86%), mainly chronic kidney failure, hearing loss and deafness.
Today, drug toxicity is managed more carefully and long-term remissions are possible. Some patients are able to lead relatively normal lives and remain in remission for 20+ years after treatment.
Vasculitis can be classified by the cause, the location, the type of vessel or the size of vessel.
- "Underlying cause". For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to inflammation of the aorta, which is an artery.) However, the causes of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides.
- "Location of the affected vessels". For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" (corresponding to systemic vasculitis) with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
- "Type or size of the blood vessels" that they predominantly affect. Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.
According to the size of the vessel affected, vasculitis can be classified into:
- Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, Temporal arteritis
- Medium vessel: Buerger's disease, Kawasaki disease, Polyarteritis nodosa
- Small vessel: Behçet's syndrome, Eosinophilic granulomatosis with polyangiitis, Cutaneous vasculitis, Henoch–Schönlein purpura, Microscopic polyannulomatosis ConditionofSome disorders have vasculitis as their main feature. The major types are given in the table below:
Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.
Furthermore, there are many conditions that have vasculitis as an accompanying or atypical feature, including:
- Rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis
- Cancer, such as lymphomas
- Infections, such as hepatitis C
- Exposure to chemicals and drugs, such as amphetamines, cocaine, and anthrax vaccines which contain the Anthrax Protective Antigen as the primary ingredient.
In pediatric patients varicella inflammation may be followed by vasculitis of intracranial vessels. This condition is called post varicella angiopathy and this may be responsible for arterial ischaemic strokes in children.
Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies. These are:
- Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome)
- Microscopic polyangiitis
If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the fetus, especially if he or she is treated before the sixteenth week of pregnancy. The fetus is at greatest risk of contracting syphilis when the mother is in the early stages of infection, but the disease can be passed at any point during pregnancy, even during delivery (if the child had not already contracted it). A woman in the secondary stage of syphilis decreases her fetus's risk of developing congenital syphilis by 98% if she receives treatment before the last month of pregnancy. An afflicted child can be treated using antibiotics much like an adult; however, any developmental symptoms are likely to be permanent.
Kassowitz’s law is an empirical observation used in context of congenital syphilis stating that the greater the duration between the infection of the mother and conception, the better is the outcome for the infant. Features of a better outcome include less chance of stillbirth and of developing congenital syphilis.
The Centers for Disease Control and Prevention recommends treating symptomatic or babies born to infected mother with unknown treatment status with procaine penicillin G, 50,000 U/kg dose IM a day in a single dose for 10 days. Treatment for these babies can vary on a case by case basis. Treatment cannot reverse any deformities, brain, or permanent tissue damage that has already occurred.
The formation of gummata is rare in developed countries, but common in areas that lack adequate medical treatment.
Syphilitic gummas are found in most but not all cases of tertiary syphilis, and can occur either singly or in groups. Gummatous lesions are usually associated with long-term syphilitic infection; however, such lesions can also be a symptom of benign late syphilis.