“itis” means inflammation and can affect any part of the eye
Inflammation can be caused by injury or microbes and there is often a genetic predisposition. If you are predisposed to inflammation there are things you can do in your lifestyle to reduce your inflammatory drive. Even with the best anti-inflammatory lifestyle, severe inflammation needs to be treated with evidence based medical interventions. Please follow this link to read about an anti-inflammatory lifestyle. It is very difficult to study the effect of lifestyle measures, particularly on rare conditions so this advice is general. It is useful to discuss with your doctor any natural or supplement treatment you may be considering, to determine if it is right/ safe for your specific condition. E.g., 1 Vitamin D can be helpful in the setting of true autoimmune diseases but can trigger or aggravate rosacea. E.g., 2 Some vitamins may reduce the beneficial effects of your immunomodulatory treatment. E.g., 3 You don’t want to improve your immune drive with immune boosters if immunity is aggravating your inflammation.
UVEITIS
WHAT IS UVEITIS?
Uveitis (pronounced “you-vee-EYE-tis”) is inflammation of the uvea. The uvea is the layer of the eye that contains blood vessels that carry oxygen to and from the eye. It includes the iris, ciliary body, and choroid.
Uveitis can be classified into different forms, depending on which part of the eye is affected by the inflammation.
Anterior uveitis (known as iridocyclitis or iritis) is inflammation of the iris, the coloured part of the eye. This is the most common form of uveitis. This condition can occur as a single episode and subside with proper treatment, it may be recurrent and affect either eye or it may become chronic.
Intermediate uveitis is inflammation of the uvea behind the lens of the eye. White blood cells within the vitreous gel of the eye cause floaters which can look like a sandstorm. Pars planitis is intermediate uveitis where an inflammatory band is seen at the bottom of the vitreous gel behind the iris, in addition to the cells in the vitreous.
Posterior uveitis (known as chorioretinitis) is inflammation of the retina and choroid layers at the back of the eye, involved in light detection.
Pan-uveitis is inflammation of all of the uvea.
WHAT CAUSES UVEITIS? WHAT ARE THE SYMPTOMS OF UVEITIS?
The different types of uveitis cause different symptoms:
Anterior Uveitis (frequently just one eye is involved)
- Pain in the eye.
- Redness of the eye around the edge of the iris.
- Blurred vision.
- Sensitivity to light (known as “photophobia”). The red eye can feel discomfort when the opposite eye is exposed to light.
Intermediate Uveitis
Posterior Uveitis
HOW IS UVEITIS DIAGNOSED?
A careful eye examination by an ophthalmologist is very important as the inflammation can only be visualised using the slit-lamp microscopes used by ophthalmologists.
Your ophthalmologist will examine the inside of your eye. Depending on the eye findings, blood tests, radiology and others may be done to help determine the diagnosis and identify safe treatments.
In approximately 40% to 60% of cases, no associated disease can be found by this kind of testing but in most cases a label can be put on the condition to help plan treatment.
WHAT IS THE TREATMENT FOR UVEITIS?
Uveitis needs to be treated as soon as possible.
Eye drops such as steroids and drops which dilate the pupil are used to reduce inflammation and pain.
More severe inflammation may require oral medication or injections.
Complications from uveitis can include:
- Glaucoma (increased pressure in the eye).
- Cataract (clouding of the eye’s lens).
- Growth of new, abnormal blood vessels
- Damage to the retina
- Damage to the optic nerve
Common types of uveitis managed by uveitis specialists (and general ophthalmologists)
- HLAB27 uveitis (often seen with Ankylosing spondylitis or reactive arthritis)
- Inflammatory bowel disease related uveitis
- Fuchs Uveitis Syndrome
- Psoriatic uveitis
- Sarcoidosis
- Shingles uveitis (Varicella Zoster Ophthalmicus)
Uncommon and rare types of uveitis managed by uveitis specialists
- Acute occult outer zonal retinopathy (AZOOR)
- Acute idiopathic blind spot enlargement (AIBSE)
- Acute macular neuroretinitis (AMN)
- Ampiginous chorioretinitis
- Behcets uveitis
- Birdshot chorioretinopathy
- Cytomegalovirus uveitis
- Intermediate uveitis
- Juvenile Idiopathic Arthritis associated uveitis
- Multifocal chroiditis and panuveitis
- Multifocal Outer Retinopathy and Retinal pigment epitheliopathy (MORR)
- Multiple Evanescent White Dot Syndrome (MEWDS)
- Panuveitis
- Punctate Inner Choroidopathy (PIC)
- Relentless placoid chorioretinitis
- Serpiginous chorioretinopathy
- Sympathetic ophthalmia
- Tattoo Associated Granulomatous Uveitis
- Toxoplasma uveitis
- Tubercular retinal vasculitis
- Tubercular serpiginous like chorioretinopathy
- Tubulointerstitial Nephritis and Uveitis (TINU)
- Vogt Koyanagi Harada Disease (VKH)
…and others
Many of these conditions have support groups run through Facebook or with their own dedicated website, so if you need somebody to share your experience with, it’s worth having a look online.
The American Academy of Ophthalmology runs an up-to-date information page called EyeWiki if you are looking for more technical information. Type in the name of your condition and then EyeWiki.
SCLERITIS
WHAT IS SCLERITIS?
Scleritis is inflammation deep within the white of the eye. The redness is a dark beefy colour. It can be diffuse (whole eye), or nodular (a red lump) and sometimes it can make the white of the eye melt away. It can affect the front or back of the eye. If it is at the back, sometimes the redness is not visible.
Scleritis is usually very painful and causes a boring ache that radiates into the brow, behind the eye and behind the ear. Typically, the pain gets worse at night and can wake people because it is quite severe. In people taking immunosuppressive medication for other conditions like Rheumatoid arthritis and Lupus, there may be no pain, and redness is the only clue that something serious is going on with the eye.
WHAT DISEASES ARE ASSOCIATED WITH SCLERITIS?
In around 50% of cases we can detect a cause through a blood test or CT scan. Commonly these show up conditions such as rheumatoid arthritis, lupus, granulomatosis with polyangiitis, ankylosing spondylitis, or sarcoidosis.
Rosacea is an under recognised trigger for scleritis. This can be detected by a good history and examination rather than by special tests.
Infections such as herpes viruses, varicella, bacteria, and fungi can cause very serious scleritis with melting of the white of the eye.
WHAT IS THE TREATMENT OF SCLERITIS?
Scleritis typically does not respond well to topical eye drops, and oral or injected medications are needed. These could include Non-steroidal anti-inflammatory medications (NSAIDS e.g. naproxen), steroids (e.g. prednisolone, triamcinolone), immunomodulatory treatments (e.g. methotrexate), or biologics (e.g. adalimumab). If an underlying immune disease is found, then that needs to be treated in order to control the scleritis. If the trigger is rosacea, then doxycycline or minocycline in conjunction with a short course of NSAIDS can be highly effective.
Infectious scleritis usually requires treatment with a combination of antimicrobials and anti-inflammatory medications.
EPISCLERITIS
WHAT IS EPISCLERITIS?
Episcleritis is inflammation of the connective tissue between the outer conjunctival membrane of the eye and the white of the eye. It can be diffuse or nodular. Typically, it is painless but it can cause a bruised feel. Attacks usually last days or a couple of weeks and do not need treatment. It is highly unusual to be able to detect a cause by doing blood tests, but a common trigger in fair skinned people is rosacea, particularly if there is inflammation of the eyelid glands (meibomitis or blepharitis).
WHAT IS THE TREATMENT OF EPISCLERITIS?
Because this condition is not sight threatening and is self-limiting, it may not need any treatment. Treating any inflammation of the meibomian glands and avoiding triggers such as eye makeup may be all that is needed. A brief course of over-the-counter NSAIDS such as Nurofen can be used to treat the bruised feel and cooled lubricant drops may be soothing. Where possible, steroid eye drops are avoided as their side effects (raised eye pressure, cataract, potentiating microbially triggered inflammation) can outweigh their limited benefits.
KERATITIS
WHAT IS KERATITIS?
Keratitis is inflammation of the cornea or clear window of the eye. It will usually cause light sensitivity, pain and blurry vision. The most common cause in a comprehensive practice is dry eye which can be a significant trigger for inflammation. Other causes are:
Types of keratitis
- Marginal keratitis means an ulcer on the cornea just inside the margin of the clear part of the eye. It is a hypersensitivity reaction commonly seen in people with rosacea. Treatment is with antibiotic and steroid drops as well as treating the rosacea if it is active.
- Contact lens keratitis (usually bacterial)– sleeping, showering or swimming in contact lenses are risk factors for this condition. Bacterial keratitis can cause significant visual loss with perforation of the eye and sometimes the need for a corneal transplant. PLEASE DO NOT swim, shower or sleep in your contact lenses! Infection related to contact lenses requires special testing and culture techniques which, in Perth can only be done at the teaching hospitals. It is best to be referred straight to your nearest teaching ophthalmology department without starting antibiotics so that the correct microbe can be identified to be sure that the right treatment is given.
- Fungal keratitis occurs after garden or agricultural contamination. Always wash your eye very well if soil or contaminated material gets in your eye. Fungal keratitis needs to be treated in a teaching hospital.
- Viral keratitis
- Herpes Simplex keratitis can take many forms including the classical dendritic ulcer.
- Varicella keratitis occurs after ophthalmic shingles infection and can require months or even years of treatment with sometimes devastating loss of vision. PLEASE prevent this by getting your SHINGLES VACCINATION if you are eligible or can afford to pay for it privately.
- Post-adenoviral keratitis can occur a couple of weeks after community acquired adenoviral conjunctivitis. A risk factor is treatment of the initial conjunctivitis with steroids so it is best to avoid this where possible.
- Autoimmune keratitis Rheumatoid arthritis and other immune disorders can be associated with melting of the cornea. This can happen when the immune disease seems under control or burnt out. A change in systemic treatment is always needed. Corneal melting can actually indicate life threatening internal inflammation. Seeing a uveitis specialist who can work with your corneal specialist, rheumatologist or immunologist has been shown in a New Zealand study to be life saving.
- There are many other types of keratitis such as allergic and immune related keratitis. The treatment can range from drops to immune modulation using tablets or injectable biologics.
CONJUNCTIVITIS
WHAT IS CONJUNCTIVITIS?
Conjunctivitis is inflammation of the surface membrane covering the white of the eye. The most common cause is viral infection which is seldom sight threatening and usually gets better by itself after 7-10 days.
Types:
- Bacterial conjunctivitis is most common in children and causes sticky crusty eyes. Usually the child will have a strong enough immune system that no antibiotics are needed and just cleaning the eye with water or a dilute bicarb soda/ dilute salty solution for a few days is enough.
- Viral conjunctivitis is highly contagious. The most common cause is adenovirus which survives for many days on contaminated surfaces. Often people don’t know where they got their infection. Antibiotics do not help and are not indicated. Chilled lubricant drops may give some relief. Viral conjunctivitis causes burning, watering and discomfort with a bright red eye. It often starts on one side and spreads to the other after a few days. Frequently there is a tender lymph node just in front of the ear. People with conjunctivitis should stay off work or school until better. It is not necessary to see a doctor or eye specialist. Bringing the virus into a waiting room should be avoided and if there is any doubt about the diagnosis, a teleconsult is the most sensible course of action. The eyes are often dry and tired for months and preservative free lubricants are needed. Sometimes viral conjunctivitis can be followed by post-adenoviral keratitis a few weeks later. This causes blurry vision and light sensitivity. An ophthalmologist can provide advice (which is typically to ride it out as it will eventually get better).
- STD conjunctivitis Sexually transmitted diseases are an emerging health threat. Gonococcal conjunctivitis is associated with a severe pussy exudate and can be sight threatening. It needs to be treated in a teaching hospital. Chlamydia conjunctivitis tends to cause a very irritated red eye which does not get better despite weeks of standard antibiotics. It requires strong oral antibiotics.
- Allergic or Papillary conjunctivitis can cause itchy or gritty eyes. People with this condition often have a history of asthma, eczema, or hay fever at some stage in their life. Initial treatment is to avoid the trigger if known. Over the counter or prescription drops such as twice daily zaditen preservative free or patanol can be tried. These are safe for prolonged use up to twice a day. Only if these do not help is it necessary to see an ophthalmologist who might recommend steroid or other treatment.
MEDICINES USED BY A UVEITIS SPECIALIST OR OCULAR IMMUNOLOGIST
Ophthalmologists are doctors (usually 6 years’ training) who have specialised in eye medicine and eye surgery (usually 4 years basic training). Uveitis specialists have done further training (usually 1-2 years) in managing ocular inflammation and infection. Just like rheumatologists and immunologists they are trained to use complex medications to dampen down immunity or inflammation as safely as possible in order to save sight. They can also do eye surgery in people with immune conditions requiring special peri-operative and intra-operative management. They know when a condition is likely to be manageable just with eye drops as well as which diseases need to be treated with stronger treatment. It can be daunting to be told that you need to take strong medication or have an operation because of inflammation in your eye. You can be assured that uveitis specialists are constantly talking to each other to work out the best way to treat these eye conditions which are often rare.
HOW DO WE KNOW THE MEDICINES ARE SAFE AND EFFECTIVE
Even though many conditions seen by uveitis specialists are rare, there is a global network of colleagues who collaborate to collect cases and run studies and trials to find out what works and check that it is safe. Important evidence from peer reviewed studies helps us to save sight and also often to save life. These are some of the studies:
The MUST study showed that after 7 years of treatment, modulating the whole immune system (systemic immunosuppression) with tablets or biologic injectables achieved superior uveitis control over steroids injected into or implanted directly into the eye.
The FAST study showed that Methotrexate and Mycophenolate had similar efficacy in a population of people with uveitis from across the globe. Methotrexate is probably slightly more effective for posterior uveitis.
The SITE study showed that over more than 10 years of systemic immunosuppression is safe and also that there is 50% risk of glaucoma after 10 years of management for uveitis.
The POINT study showed that injecting or implanting steroids inside the eye works better than injecting them round the back of the eyeball.
The MERIT showed that when injected into the eye, dexamethasone (steroid) works better than ranibizumab which works better than methotrexate. The latter two can still be used if steroids are likely to be too risky in a particular eye.
We also get information from registries such as the Fight Uveitis Blindness registry and various population registries e.g., In Denmark, a country of around 5 million people, there are about 5000 people taking methotrexate. These people did fine during the COVID pandemic and methotrexate did not increase their risks from COVID-19.
WHAT DRUGS MIGHT MY UVEITIS SPECIALIST RECOMMEND
Common medications include:
Steroids such as prednisolone which can be given orally or intravenously: these are used when quick control is needed (putting out the fire). They have too many side effects to be used at high doses in the long term. Often usually a different slower acting steroid sparing medication will be started at the same time while steroid treatment is tapered. Side effects include weight gain, diabetes, bone thinning, high blood pressure, high eye pressure, and cataracts.
Methotrexate can be given orally or injected under the skin once a week. It has been around for decades so there is a lot of experience with long-term use in adults with conditions like rheumatoid arthritis and children with juvenile arthritis. It has also proven highly effective for many kinds of uveitis. Because methotrexate can be used as a chemo drug it can be worrying to be asked to take it for an eye condition. In chemo it is used at much higher and more frequent doses than we need for eye conditions and it has proven very safe when used at lower doses. Some people find it makes them tired or a bit nauseous until they get used to it but many people have no side effects at all. We always check regularly that it is not affecting the liver or blood count (usually 3 monthly in stable patients). The once a week dosing makes it very convenient and easy to use.
Mycophenolate is an oral medication which has to be taken twice a day. Most people tolerate it with no side effects at all but some people can get nausea or diarrhoea. We always check regularly that it is not affecting the liver or blood count (usually 3 monthly in stable patients).
Azathioprine is an oral medication daily. Most people tolerate it well but some people can get nausea or gut disturbances. It is a bit weaker than methotrexate and mycophenolate in controlling uveitis but its big advantage is that it is safe in pregnancy. We always check regularly that it is not affecting the liver or blood count (usually 3 monthly in stable patients).
Cyclosporine is an oral medication which is taken twice a day. It can be useful for people who have been nauseous on methotrexate or mycophenolate and can be used to allow lower dosing of these medications. It can cause high blood pressure and the kidneys need to be monitored regularly while on it.
Adalimumab is an injectable biologic which works against the inflammatory molecule TNF. It is usually self-injected every fortnight and has very few side effects other than the inconvenience of having keep it cold with a fridge pack if travelling.
Because all of these dampen down the immune system there are a few pieces of important advice people using such medications should follow:
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- Avoid exposing yourself to infection when you can e.g., wear a mask when using potting mix, clean contaminated wounds well, stay away from people who are sick.
- Make sure all your vaccinations are up to date, especially Shingrix (the shingles vaccination), the flu vaccination, and COVID vaccinations.
- If you are admitted to hospital because you are ill, tell your doctors what immunosuppression you are taking. Sometimes you may need to omit a dose. Often you can just keep going with your usual medication but it is likely you will need antibiotics if a bacterial infection is considered likely.
- Keep up to date with your skin checks. The immune system is needed for getting rid of skin cancers. There may be a higher risk of developing skin cancers if you have had a lot of sun exposure. When these medicines are used for eye disease they do not seem to increase the risk of non-skin cancers as has been the case when they are used for non-ocular immune diseases.