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In very severe cases of necrotizing scleritis, eye surgery must be performed to repair damaged corneal tissue in the eye and preserve the patient's vision. For less severe cases, nonsteroidal anti-inflammatory drugs, such as ibuprofen, are prescribed for pain relief. Scleritis itself is treated with an oral medication containing corticosteroids and an eye solution. In some cases, antibiotics are prescribed. Simply using eye drops will not treat scleritis. In more aggressive cases of scleritis, chemotherapy (such as systemic immunosuppressive therapy with such drugs as cyclophosphamide or azathioprine) may be used to treat the disease. If not treated, scleritis can cause blindness.
Often, treatment is not necessary, because episcleritis is a self-limiting condition. Artificial tears may be used to help with irritation and discomfort. More severe cases can be treated with either topical corticosteroids or oral non-steroidal anti-inflammatory drugs.
Ketorolac, a topical NSAID, may be used, but it is not more effective than artificial tears and it causes more side effects.
The acute uveitis phase of VKH is usually responsive to high-dose oral corticosteroids; parenteral administration is usually not required. However, ocular complications may require an subtenon or intravitreous injection of corticosteroids or bevacizumab. In refractory situations, other immunosuppressives such as cyclosporine, or tacrolimus, antimetabolites (azathioprine, mycophenolate mofetil or methotrexate), or biological agents such as intravenous immunoglobulins (IVIG) or infliximab may be needed.
One treatment used is polyhexamethylene biguanide, PHMB.
Propamidine isethionate has also shown some effectiveness.
Another possible agent is chlorhexidine.
Keratoplasty may sometimes be required.
A combined regimen of propamidine, miconazole nitrate, and neomycin has also been suggested.
A recent Cochrane review found one study that compared the effectiveness of chlorhexidine eye drops against PHMB eye drops, for eyes with "Acanthamoeba" keratitis. The differences between treatments were not statistically significant; the review found that 86% of eyes treated with chlorhexidine eye drops reported a resolution of infection, compared to 78% of eyes treated with PHMB eye drops. The study also found that 71% of eyes treated with chlorhexidine eye drops reported improved visual acuity after treatment, compared to 57% of eyes in the PMGB group; these results were also not significant.
Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment. Inner ear symptoms usually respond to corticosteroid therapy within weeks to months; hearing usually recovers completely. Chronic eye effects such as cataracts, glaucoma, and optic atrophy can occur. Skin changes usually persist despite therapy.
According to the American Optometric Association, the following steps can be taken to prevent "Acanthamoeba" keratitis:
- Always wash and dry your hands before handling contact lenses, ordinary water should never come in contact with your lenses.
- Rub and rinse the surface of the contact lens before storing.
- Use only sterile products recommended by your optometrist to clean and disinfect your lenses. Saline solution and rewetting drops are not designed to disinfect lenses.
- Avoid using tap water to wash or store contact lenses.
- Contact lens solution must be discarded upon opening the case, and fresh solution used each time the lens is placed in the case.
- Replace lenses using your doctor’s prescribed schedule.
- Do not sleep in contact lenses unless prescribed by your doctor and never after swimming.
- Never swap lenses with someone else.
- Never put contact lenses in your mouth.
- See your optometrist regularly for contact lens evaluation.
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.
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.
Episcleritis is inflammation of the episclera, a less serious condition that seldom develops into scleritis.
The standard treatment for GPA is cyclophosphamide and high dose corticosteroids for remission induction and less toxic immunosuppressants like azathioprine, leflunomide, methotrexate or mycophenolate mofetil. Trimethoprim/sulfamethoxazole may also help prevent relapse. Rituximab may be substituted for cyclophosphamide in inducing remission.
A systematic review of 84 trials examined the evidence for various treatments in GPA. Many trials include data on pooled groups of people with GPA and microscopic polyangiitis. In this review, cases are divided between localised disease, non-organ threatening, generalized organ-threatening disease and severe kidney vasculitis and immediately life-threatening disease.
- In generalised non-organ-threatening disease, remission can be induced with methotrexate and steroids, where the steroid dose is reduced after a remission has been achieved and methotrexate used as maintenance.
- In case of organ-threatening disease, pulsed intravenous cyclophosphamide with steroids is recommended. Once remission has been achieved, azathioprine and steroids can be used to maintain remission.
- In severe kidney vasculitis, the same regimen is used but with the addition of plasma exchange.
- In pulmonary haemorrhage, high doses of cyclophosphamide with pulsed methylprednisolone may be used, or alternatively CYC, steroids, and plasma exchange.
Therapy for GPA and MPA has two main components: induction of remission with initial immunosuppressive therapy, and maintenance of remission with immunosuppressive therapy for a variable period to prevent relapse.
The mainstay of treatment for granulomatosis with polyangiitis (GPA) is a combination of corticosteroids and cytotoxic agents.
- Medications
- Side effect treatments
- Plasma exchange
- Kidney transplant
There are no prospective randomized controlled trials studying therapies for relapsing polychondritis. Evidence for efficacy of treatments is based on case reports and series of small groups of patients.
For mild cases limited to joint pain or arthritis, oral nonsteroidal anti-inflammatory drugs (NSAIDs) may be used. Other treatments typically involve medications to suppress the immune system. Corticosteroids are frequently used for more serious disease. Steroid-sparing medications such as azathioprine or methotrexate may be used to minimize steroid doses and limit the side effects of steroids. For severe disease cyclophosphamide is often given in addition to high dose intravenous steroids.
Ulcerative colitis can be treated with a number of medications, including 5-ASA drugs such as sulfasalazine and mesalazine. Corticosteroids such as prednisone can also be used due to their immunosuppressive and short-term healing properties, but because their risks outweigh their benefits, they are not used long-term in treatment. Immunosuppressive medications such as azathioprine and biological agents such as infliximab and adalimumab are given only if people cannot achieve remission with 5-ASA and corticosteroids. Such treatments are used less commonly due to their possible risk factors, including but not limited to increased risk of cancers in teenagers and adults, tuberculosis, and new or worsening heart failure (these side effects are rare). A formulation of budesonide was approved by the FDA for treatment of active ulcerative colitis in January 2013. The evidence on methotrexate does not show a benefit in producing remission in people with ulcerative colitis. Off-label use of drugs such as ciclosporin and tacrolimus has shown some benefits. Fexofenadine, an antihistamine drug used in treatment of allergies, has shown promise in a combination therapy in some studies. Opportunely, low gastrointestinal absorption (or high absorbed drug gastrointestinal secretion) of fexofenadine results in higher concentration at the site of inflammation. Thus, the drug may locally decrease histamine secretion by involved gastrointestinal mast cells and alleviate the inflammation.
Sulfasalazine has been a major agent in the therapy of mild to moderate ulcerative colitis for over 50 years. In 1977, Mastan S. Kalsi "et al." determined that 5-aminosalicylic acid (5-ASA and mesalazine) was the therapeutically active component in sulfasalazine. Since then, many 5-ASA compounds have been developed with the aim of maintaining efficacy but reducing the common side effects associated with the sulfapyridine moiety in sulfasalazine.
Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology, pediatric rheumatology, and pediatric infectious disease specialists (although no specific infectious agent has been identified as yet). Treatment should be started as soon as the diagnosis is made to prevent damage to the coronary arteries.
Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. In rare cases, a third dose may be given to the child. IVIG by itself is most useful within the first seven days of onset of fever, in terms of preventing coronary artery aneurysm.
Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as IVIG. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for two months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye's syndrome. Because children with Kawasaki disease will be taking aspirin for up to several months, vaccination against varicella and influenza is required, as these infections are most likely to cause Reye's syndrome.
High-dose aspirin is associated with anemia and does not confer benefit to disease outcomes.
Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. Additionally, corticosteroid use in the setting of Kawasaki disease is associated with increased risk of coronary artery aneurysm, so its use is generally contraindicated in this setting. In cases of Kawasaki disease refractory to IVIG, cyclophosphamide and plasma exchange have been investigated as possible treatments, with variable outcomes.
Many individuals have mild symptoms, which recur infrequently, while others may have persistent problems that become debilitating or life-threatening.
It is the name given to the localised bulge in limbal area, lined by the root of the iris. It results due to ectasia of weak scar tissue formed at the limbus, following healing of a perforating injury or a peripheral corneal ulcer. There may be associated secondary angle closure glaucoma, may cause progression of the bulge if not treated. Defective vision occurs due to marked corneal astigmatism. Treatment consists of localised staphylectomy under heavy doses of oral steroids.
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.
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
Those with conjunctivitis may report mild irritation or scratchiness, but never extreme pain, which is an indicator of more serious disease such as keratitis, corneal ulceration, iridocyclitis, or acute glaucoma.
A staphyloma is an abnormal protrusion of the uveal tissue through a weak point in the eyeball. The protrusion is generally black in colour, due to the inner layers of the eye. It occurs due to weakening of outer layer of eye (cornea or sclera) by an inflammatory or degenerative condition.
It may be of 5 types, depending on the location on the eyeball ("bulbus oculi").
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.
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.
With early treatment, rapid recovery from the acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited ("i.e." the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement.
Laboratory evidence of increased inflammation combined with demographic features (male sex, age less than six months or greater than eight years) and incomplete response to IVIG therapy create a profile of a high-risk patient with Kawasaki disease. The likelihood that an aneurysm will resolve appears to be determined in large measure by its initial size, in which the smaller aneurysms have a greater likelihood of regression. Other factors are positively associated with the regression of aneurysms, including being younger than a year old at the onset of Kawasaki disease, fusiform rather than saccular aneurysm morphology, and an aneurysm location in a distal coronary segment. The highest rate of progression to stenosis occurs among those who develop large aneurysms. The worst prognosis occurs in children with giant aneurysms. This severe outcome may require further treatment such as percutaneous transluminal angioplasty, coronary artery stenting, bypass grafting, and even cardiac transplantation.
A relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires rehospitalization and retreatment. Treatment with IVIG can cause allergic and nonallergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of nontreatment. Also, evidence indicates Kawasaki disease produces altered lipid metabolism that persists beyond the clinical resolution of the disease.