Lupus

Thursday, September 13, 2012

Ocular manifestations of SLE

     Lupus can cause complications to any organ, or body system.  There are many ways in which lupus can affect the eyes.  Scleritis, which is a disease which causes inflammation to the whites of the eyes is one of the ways in which lupus can affect the eyes. Medications for lupus can also cause issues with this organ.  Many of the complications have the ability to cause blindness, therefore, if you have any eye pain, redness, light sensitivity or vision changes see your doctor promptly. 

Scleritis – Pictures, Symptoms, Causes, Diagnosis, Treatment

Written by Dr.Mary

What is Scleritis?

This is an inflammatory disease which is serious and which affects the white outer coating of the eye, known as the sclera – normally referred to as the whites of the eye. This is the tough, white tissue which gives the eye its shape and which protects the eye. This is a chronic and painful disease and approximately 50% of cases are contracted along with the association of other disease including granulomatosis or rheumatoid arthritis. It can also be attained thru disorders of menstruation and is fairly common among young women. This is a potentially blinding inflammatory illness that is categorized by swelling as well as cellular intrusion of the scleral as well as the episcleral tissues.

The disorder occurs most frequently in individuals who are 30 to 60 years of age – it is very rare to occur in children. If scleritis is left untreated it can spread to the surrounding features in the eye and damage the eye itself, causing problems in vision.

Scleritis Symptoms

The major symptoms of scleritis are redness in the whites of the eye as well as pain. These symptoms normally gradually develop but eventually become severe. The redness can become a very intense purple. The pain radiates from the eye to adjacent areas of the face and head. Less commonly, the eye can become teary as well as very sensitive to light and the individual can lose some vision.
Symptoms include:
  • Blurred vision
  • Eye pain and tenderness – severe
  • Red patches on the normally white part of the eye
  • Sensitivity to light – very painful
  • Tearing of the eye
The symptoms of scleritis may affect only one or both eyes. A rare form of this disease causes no eye pain or redness. If not diagnosed and treated fairly promptly, complications can develop which are serious. These include puncture of the eyeball, loss of vision as well as loss of the eyeball.

Scleritis Causes

There are many causes of scleritis. The causes of this disorder which are most common are listed below. These causes affect more than approximately one million individuals in the United States:
  • Rheumatoid arthritis
  • SLE
The following causes of scleritis are conditions or diseases that affect more than 200,000 people but less than 1 million individuals in the United States:
  • HIV
  • Inflammatory bowel disease
  • Wegnener’s granulomatosis – scleritis
The following causes of scleritis are considered very rare and appear in the population at a rate of substantially less than 200,000 individuals in the United States.
  • Syphilis
  • Sarcoidosis
  • Varicella-zoster virus
  • Tuberculosis
The following causes have no stats on the number of individuals in the United States who are affected:
  • Polyarthritis nodosa
  • Herpes simplex
  • Polychondritis
  • Rheumatoid arthritis
  • Relapsing polychondritis
  • Rheumatoid disease
  • SLE
  • Vasculitis
  • Urate crystal arthropathy

Scleritis Diagnosis

When you visit the eye doctor with symptoms suggesting scleritis, he/she will ask you about your medical history as well as conduct a thorough exam. Additionally, because of the association between scleritis and other medical diseases, the doctor may suggest a very comprehensive medical exam, including blood test and other tests and evaluations. Computed tomography or CT scanning, ultrasound, magnetic resonance imaging MRI as well as a biopsy can be used to eliminate other causes for the symptoms.

Scleritis Treatment

Topical Anti-inflammatory medications

Individuals with scleritis usually do not respond to topical medications such as NSAIDs or corticosteroids. But there are a minority of individuals who are persuaded that an eye drop for example prednisolone acetate 1% is helpful and this allows them to reduce or stop all together any oral medications.

Oral NSAIDS

Many individuals with scleritis do well on an oral NSAID, and these are generally the first treatment for this condition.

Oral corticosteroids

These medications are widely used in the management of scleritis and are the first treatment for necrotizing inflammation. Dosages are individualized on the basis of tolerance as well as response. Individuals on corticosteroids need to be carefully watched with regards to blood sugar, mood, weight and bone density.

Immunosuppressive drugs

Approximately 25% of individuals with scleritis require treatment with corticosteroid-sparing immunosuppressive medications to achieve any long-term control of this disease. Drugs under this classification include:
  • Methotrexate
  • Mycophenolate
  • Azathioprine
  • Ciclosporin
  • Mofetil
The choice of agent depends on the presence of any underlying disease as well as alcohol intake, liver function as well as blood pressure.

Surgical management

Surgical management in a small number of individuals with necrotizing scleritis, it may be necessary for surgery to preserve the integrity of the eye globe. In these cases, the outcome can be improved by controlling rapidly inflammation with aggressive immunosuppressive therapy.

Scleritis Pictures

ScleritisScleritisScleritisScleritisScleritis

Ocular manifestations of systemic lupus erythematosus

Abstract http://rheumatology.oxfordjournals.org/content/46/12/1757.full

Ocular manifestations of lupus are fairly common, may be the presenting feature of the disease and can be sight-threatening. Almost any part of the eye and visual pathway can be affected by inflammatory or thrombotic processes. Ocular pain and visual impairment require urgent assessment by an ophthalmologist. Infection should be excluded. Optic neuritis and ischaemic optic neuropathy may be difficult to distinguish. Scleritis and severe retinopathy require systemic immunosuppression but episcleritis, anterior uveitis and dry eyes can usually be managed with local eye drops. Vaso-occlusive disease, particularly in the presence of antiphospholipid antibodies, requires treatment with anticoagulation and proliferative retinopathy is treated with laser therapy. Hydroxychloroquine rarely causes ocular toxicity at doses under 6.5mg/kg/day. When this has occurred, it has been associated with more than 5 years of drug exposure.

Key words

Systemic lupus erythematosus (SLE) is a chronic, autoimmune, multisystem disease which may affect the eyes and/or visual system in up to a third of patients. These ocular manifestations cause significant morbidity in their own right, but can also be a useful indicator of underlying systemic disease activity. Although early recognition and treatment have led to a reduction in severe ocular complications, ocular involvement in SLE is still a potentially blinding condition.

Methods

In addition to the authors’ personal knowledge of the subject, we performed a comprehensive literature search using PubMed, medline and dialog datastar from 1966 to present using key words lupus, SLE, eye, orbit, conjunctiva, cornea, glaucoma, iridocyclitis, retina, choroid, neuro ophthalmology and treatment. We also mined the bibliographies of the included publications for additional relevant publications. It should be noted that there is a paucity of controlled studies addressing the prevalence and treatment of ophthalmic manifestations of SLE.

Pathophysiology of ocular disease

SLE may cause ocular disease by a number of mechanisms including immune complex deposition and other antibody related mechanisms, vasculitis and thrombosis. Immune complex deposition has been identified in blood vessels of the conjunctiva, retina, choroid, sclera, ciliary body, in the basement membranes of the ciliary body and cornea, in the peripheral nerves of the ciliary body and conjunctiva [1]. Antibody dependent cytotoxicity may cause retinal cell death and demyelination of the optic nerve. Pathogenic circulating antibodies include anti-phospholipid antibodies (APA) and antineuronal antibodies. Similar mechanisms centred on the lacrimal gland may result in secondary Sjogren's syndrome with consequent dry eyes (keratoconjunctivitis sicca) due to inadequate tear production; this is in marked contrast to most cases of dry eyes in the general population where it is primarily a problem of disturbance of the lipid layer of the tear film resulting in increased tear evaporation.

Diagnosis and assessment of disease activity

The diagnosis of SLE is essentially clinical and is generally based on the presence of four or more of the 11 features listed by the American College of Rheumatology classification criteria (ACR/ARA) [2]. The course of disease is highly variable with many patients having remissions between exacerbations. Assessment of disease activity by a validated activity index helps in monitoring and provides a more reliable basis for therapeutic intervention. Ophthalmic problems may be an important part of overall disease activity, and are thus featured in the latest version of the British Isles Lupus Assessment Group index of disease activity (BILAG 2004) [3].

Presentation of ophthalmic disease

Ocular manifestations in SLE are fairly common, potentially sight threatening and may be the presenting feature of their disease [45]. SLE may affect almost any part of the eye and visual pathway. Additionally drugs used in the treatment of SLE may cause ocular problems such as cataract or retinopathy.
The patient will usually be aware that there is an ‘eye problem’, and will report it to their rheumatologist (or General Practitioner). It is therefore important that the implications of these symptoms are recognized and appropriate help is sought. In general terms, pain (often accompanied by visible inflammation or redness) usually indicates significant external/anterior segment disease, whereas problems with vision (blurring, distortion, double vision usually indicates posterior segment/neuro-ophthalmic disease (Tables 1 and 2). All such complaints warrant urgent referral to an ophthalmologist for more detailed assessment.
View this table:
TABLE 1.
Causes of red eye in SLE
View this table:
TABLE 2.
Causes of loss of vision in SLE

External eye disease

Eyelid disease

SLE (and discoid lupus erythematosus) can present with a discoid lupus-type rash over the eyelids. These discrete raised scaly lesions must be distinguished from the much more common chronic blepharitis (inflammation of lid margins). These lesions usually respond well to systemic but not topical anti-inflammatory therapy [6].

Lacrimal system disease

Dry eye syndrome (keratoconjunctivitis sicca) is the most common ocular feature of SLE (around a third of patients) and is often associated with secondary Sjögren's syndrome [78]. Usually, symptoms are relatively mild (irritation, redness) but severe pain and visual loss may occur. A reduced tear film and corneal changes are evident on slit-lamp examination. Tear production can be assessed by the Schirmer test which measures the wetting of a test strip hung over the lower lid for a period of 5 min.
Milder forms of dry eye can be treated with artificial tear drops. The choice of treatment depends on balancing the disadvantages of frequent instillation required for low viscosity preparations (such as hypromellose) with the blurred vision caused by more viscous preparations (such as liquid paraffin). Often a medium viscosity drop (e.g. a carbomer) or a combination (low viscosity during the day, high viscosity at night) is preferred. In more severe cases, drainage of tears may be reduced by temporary or permanent occlusion of the puncta.

Orbital disease

Rare ocular presentations include orbital masses, periorbital oedema, orbital myositis, panniculitis, acute orbital ischaemia and infarction are rare presentations of SLE. A biopsy is often necessary to confirm the diagnosis. Treatment is with systemic immunosuppression [9–12].

Anterior segment disease

Corneal disease

Although the most common, the changes of dry eye syndrome are not the only corneal manifestation of SLE. Recurrent corneal erosions (large breaks in the corneal epithelium) typically present as a painful watery eye that comes on at waking and improves over the course of the day. Another corneal presentation is punctate epithelial loss. This may respond to systemic antimalarials suggesting that this may be an autoimmune rather than a dry eye phenomenon [13]. Peripheral ulcerative keratitis is rare and is an ominous marker of the presence of active systemic vasculitis [14]. Acute unilateral corneal stromal infiltration and oedema has been reported and responds rapidly to topical corticosteroid therapy.

Episcleral and scleral disease

Episcleritis (superficial) and scleritis (deeper inflammation of the sclera) are both seen in SLE, and may be the presenting feature of the disease [15]. Episcleritis usually presents with mild, if any, irritation and redness due to injection of the superficial blood vessels. Since only the superficial blood vessels are affected, a drop of phenylephrine (e.g. 2.5%) will cause visible blanching of the vessels, so confirming the diagnosis. Episcleritis is usually self-limiting.
Scleritis is much more painful, may be sight threatening and requires urgent assessment by an ophthalmologist. The pain is so severe that it can wake the patient from sleep; it may be described as an ‘ache’ or ‘boring’ and may be generalized to the whole eye or the side of the face. In anterior scleritis there is redness due to injection of the deeper episceral vessels (which do not blanch on the phenylephrine test); the sclera itself is avascular. The redness may be concealed under the upper lid so it is worth examining this area by lifting the lid, whilst asking the patient to look down. It is usually unilateral and is not associated with discharge. Anterior scleritis may be diffuse or nodular in distribution. Rarely it may result in significant destruction (necrotizing scleritis) leaving an area of scleral thinning (Fig. 1). Posterior scleritis does not cause a red eye (unless it extends anteriorly) but may cause visual problems, with blurring, change in refraction and double vision [16]. Although features may be present on fundoscopy (oedema, choroidal detachments, exudative retinal detachments), the diagnosis is most easily confirmed on B-scan ultrasonography.
FIG. 1.
Necrotizing anterior scleritis resulting in scleral thinning. Areas of scleral thinning appear dark due to visualization of the underlying uveal tissue.
Episcleritis does not usually require treatment, although artificial tears may be soothing. Suspected scleritis should be referred to an ophthalmologist. The presence of scleritis may indicate activity of the underlying disease and requires systemic therapy, ranging from non steroidal anti-inflammatory drugs like flurbiprofen to corticosteroids and other immunosuppressive agents (e.g. cyclophosphamide, azathioprine, methotrexate, ciclosporin or mycophenolate).

Other anterior segment complications

Conjunctival inflammation is uncommon. It presents with irritation, redness and may be associated with lid follicles.
Anterior uveitis (intraocular inflammation of the anterior part of the eye) seldom occurs in isolation. It is more commonly associated with scleritis or posterior intraocular inflammation. It is rarely of sufficient severity to be associated with a hypopyon.

Posterior segment disease

Retinal disease

Retinal disease affects around 10% of SLE patients, reflecting a reduction in frequency associated with improved control of systemic disease. Mild retinopathy may be asymptomatic but more severe disease may cause loss of vision, field defects, distortion or floaters. Such visual symptoms are therefore an indication for urgent ophthalmic review. The retinal signs often parallel the severity of systemic inflammation, and may indicate inadequate control of the systemic disease [1718]. The presence of APA is associated with more severe retinopathy and vascular occlusions [19].
Mild lupus retinopathy consists of cotton–wool spots, perivascular hard exudates, retinal haemorrhages and vascular tortuosity [20] (Figs 2 and3). Moderately severe cases may also have focal or generalized arteriolar constriction and venous tortuosity. There are some similarities to both hypertensive retinopathy and diabetic retinopathy; when these conditions occur concurrently, disease monitoring and treatment can be challenging. At the severe end of the spectrum there is occlusion of retinal arterioles and consequent retinal infarction, termed vaso-occlusive retinopathy or ‘retinal vasculitis’ [2122]. Proliferative retinopathy may occur in up to 72% of such cases, often with ensuing vitreous haemorrhage, retinal traction and retinal detachment (Figs 4 and 5). Other retinal presentations include large vessel occlusions (central and branch retinal vein occlusions, central and branch retinal arteriole occlusions) that are more common in the presence of APA (Fig. 6), pigmentary changes (pseudo-retinitis pigmentosa) and exudative retinal detachments secondary to choroidal disease. In the immunosuppressed state, rare retinal infections may occur: retinal necrosis due to herpes simplex, varicella zoster (Fig. 7) and cytomegalovirus are all reported.
FIG. 2.
Acute lupus retinopathy with cotton wool spots, haemorrhages, arterial narrowing, venous dilation and tortuosity.
FIG. 3.
Fundus fluorescein angiography of a patient with acute lupus retinopathy demonstrating capillary ‘drop-out’, vessel wall staining and leakage. Phases (i) early, (ii) arteriovenous and (iii) late.
FIG. 4.
Disc new vessels (proliferative retinopathy) in a patient with lupus vaso-occlusive retinopathy.
FIG. 5.
New vessels elsewhere (proliferative retinopathy) in a patient with lupus vaso-occlusive retinopathy. Images: (i) suspicious fundal appearance, (ii) new vessels leaking on late phase of fundus fluorescein angiogram, (iii) vessels regressed after laser treatment (photocoagulation).
FIG. 6.
Branch retinal arteriole occlusion in a patient with SLE and antiphospholipid syndrome.
FIG. 7.
Acute retinal necrosis secondary to varicella zoster virus in a patient with SLE.
The mainstay of treatment for significant retinal disease is systemic immunosuppression, but laser therapy and anti-coagulation also have a role. Initial treatment is usually with oral corticosteroids (e.g. prednisolone 1 mg/kg/day), but may be preceded by intravenous methylprednisolone (e.g. 500 mg–1 g daily for 3 days). This is then supplemented with, or replaced by, other immunosuppressive agents as part of a steroid-sparing strategy or for resistant disease. In unilateral or asymmetric disease, regional corticosteroid injections are sometimes used in addition.
In the presence of significant vaso-occlusive disease (particularly when APA are present), anti-coagulation and the addition of low dose acetylsalicylic acid may be beneficial. Proliferative retinopathy usually requires treatment with laser (panretinal photocoagulation) akin to the treatment of proliferative diabetic retinopathy [23].

Choroidal disease

Choroidal disease is less common than retinopathy but is probably underdiagnosed as a cause of visual loss in SLE. Angiography of the fundus with fluorescein and indocyanine green often demonstrates disease not detectable on clinical examination. This may include leakage into uni/multifocal retinal detachments and choroidal ischaemia. Other complications include choroidal effusions (which have been reported to cause secondary angle closure [24]), choroidal infarction and choroidal neovascular membranes [25]. In the immunosuppressed state, rare choroidal infections may occur: both a nocardia choroidal abscess [26] and tuberculous granulomas [27] with associated pulmonary disease have been reported.
Treatment is with systemic immunosuppression. However, in some patients, corticosteroids may themselves induce central serous chorioretinopathy, in which case alternative agents should be used [28]. Pulsed methylprednisolone and cyclophosphamide have been reported to be effective in treating bilateral exudative retinal detachment secondary to ischaemic choroidopathy [29].

Neuro-ophthalmic disease

Optic nerve disease

Optic nerve disease occurs in around 1% of patients with SLE [30–32], and includes optic neuritis and ischaemic optic neuropathy (anterior or posterior). Optic neuritis presents acutely with unilateral loss of vision associated with pain that is worse with eye movements. In the absence of other features of SLE, it can be very difficult to distinguish this from the typical optic neuritis of demyelinating disease [32–34]. However, the prognosis is worse in SLE associated optic neuritis, with more than half having a persistent central scotoma and progressing to optic atrophy. Pathological studies demonstrate infarction of the optic nerve secondary to extensive arteriolar fibrinoid necrosis [35]. Acute optic neuritis may also be bilateral and associated with transverse myelopathy.
In contrast to optic neuritis, optic neuropathy in SLE typically presents with bilateral, acute painless loss of vision associated with an altitudinal or arcuate field defect, with or without optic disc swelling. This is due to occlusion of the small vessels of the optic nerves, which leads to demyelination in mild cases or axonal necrosis in more severe cases. Unilateral optic neuropathy appears to reflect a ‘focal’ thrombotic event and is associated with the presence of APA. Bilateral optic neuropathy that improves with immunosuppressive treatment is suggestive of a more generalised CNS vasculitic pathology [3236].
Visual prognosis following optic neuropathy is generally poor, although good outcomes have been reported. Recurrence usually worsens the prognosis. Occasional improvement following early treatment with corticosteroids or pulsed cyclophosphamide have been reported [3031], with some patients needing anti-coagulation in addition to immunosuppression.

Cranial nerve disease and other disorders of ocular motility

Ocular motor abnormalities are not uncommon in SLE with one series reporting a rate of 29.2% [37]. Diplopia may be transient and indeed is not always present. The motility abnormalities usually arise from vasculitic or ischaemic events within the brainstem and are frequently associated with both cranial nerve and long tract signs.
Occasionally disorders of conjugate gaze such as internuclear ophthalmoplegia (unilateral [38] or bilateral [39]) and one a half syndrome (internuclear ophthalmoplegia with ipsilateral horizontal gaze palsy [40]) are seen. Other reported complications include nystagmus and Miller Fisher syndrome [41].

Other neuro-ophthalmic manifestations

Pupillary abnormalities such as light near dissociation (reduced pupillary light reflex but preserved near reflex), Horner's syndrome [37], Adie's pupil and abnormal pupillary reflexes have all been described in SLE. Blepharospasm is also reported and may be troublesome.
Retrochiasmal disease can also present with headache, lethargy, dizziness, alterations in memory and consciousness, seizures, other cranial nerve palsies, ataxia, papilloedema, amaurosis fugax (transient monocular blindness), nystagmus, field defects, cortical blindness and coma. Transient monocular blindness was observed 11 times more commonly among patients with SLE than the normal population [42]. Idiopathic intracranial hypertension has been reported in both children and adults with SLE, and may be the presenting feature of the disease [43,44].

Ophthalmic disease and the role of anti-phospholipid antibodies

The presence of APA is associated with vaso-occlusive disease (both retinal and CNS) in SLE [4546]. Interestingly retinal vascular occlusions and even a similar retinopathy may also be seen in primary anti-phospholipid syndrome. In general, the presence of APA is linked to focal thrombotic events that may prompt the use of anti-coagulation or low dose aspirin in addition to immunosuppression.

Ophthalmic disease in drug induced lupus

Ocular complications are rare in drug-induced lupus, although retinal vasculitis and occlusive disease have been reported in hydralazine and procainamide induced lupus syndrome.

Ophthalmic disease as a side-effect of treatment

The agents used in the treatment of SLE can themselves cause significant ophthalmic morbidity. Corticosteroids are commonly used in SLE and may cause cataract formation. Although steroid induced glaucoma does occur with patients taking oral corticosteroid, it is more frequent in those patients using topical corticosteroids. The introduction of other immunosuppressive agents in steroid-sparing regimes has resulted in reduced corticosteroid exposure for most patients.
Other immunosuppressive agents are usually more costly, have their own side-effects and need careful monitoring. Overwhelming septic cavernous sinus thrombosis has been reported after a combination of high dose steroid and intravenous cyclophosphamide therapy for lupus nephritis [47].
The aminoquinolones, chloroquine and, to a lesser extent, hydroxychloroquine can cause reversible visually insignificant changes in the cornea (vortex keratopathy) and, more importantly, an irreversible sight-threatening maculopathy. Initial changes are subtle (loss of foveal reflex and a fine granular appearance) and often asymptomatic, but can progress to a ‘bull's eye’ maculopathy and even generalized atrophy of the retina and optic nerve [48]. This retinopathy may continue to progress despite cessation of the drug. Although both drugs can cause identical changes the risks are much lower with hydroxychloroquine, particularly at recommended doses of up to 6.5 mg/kg/day [4950]. Below this level, hydroxychloroquine, toxicity is extremely rare. One prospective cohort study of 400 patients receiving long-term hydroxycholoroquine of up to 6.5 mg/kg/day found only two patients to be affected, in both cases only after 6 yrs of treatment [51]. Indeed Lee [52] estimated that at these recommended levels there have been only 20 affected cases in over a million patients receiving the drug; all 20 cases had been taking the drug for over 5 yrs.
In the UK, the Royal College of Ophthalmologists (in conjunction with representatives from the Royal College of Physicians Rheumatology Committee, the British society of Rheumatology and the British Association of Dermatologists) have advised that the prescribing rheumatologist should carry out the baseline assessment of lean body weight (if overweight), renal and liver function, asking about any visual impairment which is not corrected by glasses and testing reading vision [49]. Any apparent visual impairment or eye disease should be first confirmed by an optometrist, and then referred on to the local ophthalmologist before starting treatment. If visual problems occur once treatment has started, patients should be advised to stop treatment, attend their optometrist and seek advice from the prescribing physician who would refer on to the ophthalmologist. Annual evaluation should be by the prescribing rheumatologist and includes enquiry about visual symptoms and measuring reading acuity [49]. In the USA, screening by an ophthalmologist is recommended for those patients on hydroxychloroquine who are at higher risk: dose >6.5 mg/kg/day, duration of treatment > 5 yrs, renal or hepatic disease, pre-existing retinal disease or age >60 yrs [50].
Chloroquine has a less clear safety profile and should be avoided where possible. All patients taking chloroquine should have regular ophthalmic examination according to locally arranged protocol.

Conclusion

Eye manifestations in SLE may be sight-threatening and can be an indicator of active systemic disease. Significant ocular pain or reduction of vision are serious symptoms requiring urgent assessment by an ophthalmologist. The serious ocular manifestations of SLE (such as scleritis and lupus retinopathy) generally require systemic immunosuppression. Future research will hopefully provide more evidence on which to base treatment choice. Early recognition by the rheumatologist, prompt assessment by the ophthalmologist and coordinated treatment strategies are key to reducing the ocular morbidity associated with this disease.
Disclosure statement: The author have declared no conflicts of interest.
Graphic
      Lupus and the Eye
      By: Paul Savage, M.D.
      http://www.bclupus.org/reference_library/Articles%20-%20Lighthouse%20&%20other%20sources/20030401-Savage%20Paul%20MD-Lupus%20and%20the%20Eye.pdf
      The immune system protects the body from microorganisms such as bacteria and viruses, but in lupus its ability to distinguish between foreign material and its own tissues is defective.  Inflammation follows immune attack on body tissues.  At its onset, lupus may involve only one organ system.  Lupus most commonly affects joints, the skin and the kidneys but the eye may also be involved.  Autoantibodies and immune complexes damage tissues and cells. The cause of lupus is unknown.
      Ocular Symptoms and Signs 
      Ocular findings in lupus are the consequence of: 
       * Manifestations of lupus 
       * Manifestations of complications of lupus 
       * Toxicity of drug treatment for lupus or its complications 
      Ocular Manifestations of Lupus
      Dry eye symptoms of discomfort, itching, gritty sensation and reflex watering may occur when the lacrimal glands that supply tears become involved.  This may occur as part of Sjogren’s syndrome or sicca syndrome, where the salivary glands are also damaged, with dry eyes and a dry mouth.  Symptoms are generally controlled with over-the-counter tear substitutes, control of ambient humidity, and barriers to evaporation of tears (glasses, side shields, goggles) and the loss of tears (minor lid surgery.)  Although there is no specific treatment, there is a study on the use of cyclosporine in the treatment of dry eye.  DHEA has also been the subject of investigation.
      The skin around the eyes, including the eyelids, may be involved with the cutaneous changes of lupus.  A rare but severe generalized skin eruption called erythema multiforme can occur.   This causes severe inflammation of the conjunctiva that is the membrane that covers the sclera (white of the eye) and lines of the eyelids.  Resultant scarring can lead to danger of exposure of the cornea (the clear window of the eye). Other external abnormalities include a red eye resulting from inflammation of the conjuctiva and episclera (deeper blood vessels) generally without significant pain, or of the sclera (white coat of the eye) with aching pain.  If not self-limiting, these generally respond to antiinflammatory drugs.  These are seen commonly as isolated conditions without underlying disease but occur in other collagen vascular diseases.  Corneal ulceration can also occur. Intraocular problems may result from retinal vasculitis.  Retinal vasculitis is an inflammation of the blood vessels that damages and may occlude the small vessels of the retina (microangiopathy).  It is a potentially serious manifestation.  Since the retina is the tissue that lines the posterior cavity of the globe and is the light-sensing layer analogous to the film in a camera, damage resulting from occlusion of its blood supply can lead to loss of vision.  Although it can be progressive over a few days, necessitating aggressive immunosuppression to prevent blindness, it appears to be uncommon.  Examination shows narrow white (sheathed) retinal vessels and “cotton wool spots” (white patches in the retina) from localized temporary swelling.  These changes may be present in the absence of any symptoms.  Ophthalmic examination of patients with lupus-like syndromes or in seronegative cases of lupus may assist in diagnostic clarification of such patients. 
      Ocular Manifestations of Complications of Lupus
      Renal
      Renal disease may cause fluid retention and swelling of the eyelids.  “Puffiness” of the eyelids may be an early sign of relapse.  Renal hypertension, where it results in hypertensive retinopathy, may show visible retinal changes similar to those seen with lupus microangiopathy but which respond to control of the blood pressure. 
      Neurological
      An increase in the pressure of the cerebrospinal fluid that bathes the central nervous systemmay mimic an intracranial tumor (pseudotumor) and may cause swelling of the optic nerves (pseudopapilledema).  These changes may have no symptoms.  Undetected and untreated, they may lead to loss of vision with symptoms only occurring late in the disease process. 
      When the brain is involved in lupus there are sometimes ocular manifestations that help to identify the location of neurologic involvement.  Optic neuritis that impairs vision is uncommon and may mimic multiple sclerosis.  Cranial nerve palsies can result in double vision (diplopia).  These findings are also uncommon and often transitory in lupus. 
      Other problems such as cerebellar dysfunction may have eye manifestations that are subordinate to the overall clinical picture.  Deficits resulting from large strokes result in lost areas in the field of vision (peripheral vision) as part of the overall presentation. 
      Vascular
      Occlusion of a vessel may occur from problems in its wall (e.g., vasculitis), its lining (e.g., artherosclerosis), and its lumen (e.g., coagulation).  There is evidence that antibodies against phospholipids (lupus anticoagulant, anticardiolipin) may be associated with coagulation and blockage of retinal vessels.  Anticoagulation in these patients aims to reduce the risk of similar future events. 
      Lupus endocarditis may rarely be a souce of embolism or rarely become infected.  Retinal signs of infected endocarditis such as white-centered hemorrhages as well as emboli in the retinal circulation can be identified if present at the time of examination. 
      Hematologic
      Anemia (chronic disease, hemolysis, cytotoxic drugs) if severe may produce a retinopathy with retinal hemorrhages.  Low platelets (thrombocytopenia) could increase bleeding during eye surgery but a bleeding tendency, unless very severe, is not usually a problem for routine eye operations such as cataract surgery. Low white cells (leukopenia) could, hypothetically, increase susceptibility to postoperative infection and may increase the predisposition to opportunistic infections of the retina in the context of severe immunosuppression and illness.  Patients on immunosuppression after renal transplantation could be similarly at risk. 
      Gastrointestinal
      Acute pancreatitis (from active lupus, corticosteroids or azathioprine) is know to cause Purtscher’s retinopathy, a multifocal vascular injury with profuse white patches in the retina (cotton wool spots).  Vision is variable affected and tends to recover. 
      Musculoskeletal
      Children with antinuclear antibodies and pauciarticular arthritis (arthritis in few joints) may suffer severe loss of vision from an associated chronic uveitis (inflammation of iris tissue) without treatment. 
      Ocular Complications of Drugs in Lupus
      Corticosteroids
      Nonsteroid anti-inflammatory drugs very rarely have serious ocular side effects but corticosteroids do.  Serious ocular complications of chronic corticosteroid therapy include cataracts (clouding of the lens of the eye) and glaucoma (a specific form of optic nerve injury most often related to abnormal intraocular pressure).  Cataracts are treated in most cases successfully with surgery.  Risk of cataract would not ordinarily be an indication to withhold steroid treatment if it is necessary.  Likewise patients who develop glaucoma have drugs, laser treatment and surgery available to reduce their intraocular pressure.  Since glaucoma is a silent disease until almost all vision is lost and the advanced damage irreversible, it is important that any patient considered for long-term treatment with steroids be under the care of an ophthalmologist.  Steroids may also cause or aggravate existing diabetes mellitus which also may cause loss of vision in the longer term. 
      In general, risks of complications of corticosteroids increase with higher doses and extended periods of treatment. 
      Antimalarial Drugs
      Antimalarial drugs have been effective in the treatment of lupus.  Hydroxycholoroquine (Plaquenil) is now the most commonly used.  Chloroquine (Aralen) has been associated with more toxicity.  At the beginning of treatment, there may be mild blurring of vision that clears spontaneously.  Antimalarial drugs may damage the macula (the most sensitive area of the retina) and cause loss of vision.  The opthalmologist will look for retinal pigmentation that indicates early damage from antimalarial drugs.  The risk of this complication is low with the lower doses of antimalarial drugs that are now used.  There are varying opinions on what constitutes appropriate follow-up.  There is a trend toward less frequent monitoring since the doses currently used to treat lupus are rarely associated with retinal damage.  Damage due to antimalarial drugs is does-related.  Most cases of retinal damage occurred in patients who had received more that 400 mg of Plaquenil or more than 250 mg of Aralen daily.  Early minimal retinal damage from Plaquenil is reported to be reversible if the drug is stopped.  However, pigmentary changes, classically a bull’s eye appearance of the macula, are irreversible.  Based on lean body weight, doses of Plaquenil below 6.5 mg/kg/day for less than six years were not reported to be associated with retinal toxicity in a series of nearly a thousand patients.  There have been insufficient data to advise on a maximum safe dose of chloroquine. Standard of care at present would be a dilated eye examination before treatment is initiated and at least annually in the absence of problems with documentation and evaluation of any existing pigmentary abnormalities in the macula.  This may include photographic documentation.  Patients can also monitor themselves with a grid of lines (Amsler grid).  This may facilitate the detection of early symptoms from retinal damage.  In addition and dependent on other findings, tests of color vision and visual fields might be appropriate. 
      Cytotoxic Drugs
      Undesirable side effects of cytotoxic drugs (cyclophosphamide, azathioprine) include an increased risk of infection with opportunistic organisms such as herpes.  Herpes zoster may affect the ophthalmic branch of the fifth cranial nerve and damage several structures of the eye. 
      In Summary
      Ocular damage in lupus may occur from the disease or its treatment.  Fortunately blindness is uncommon.  Eye examination may reveal unsuspected complications such as glaucoma from steroids, macular damage from antimalarial drugs, pseudotumor and pseudopapilledema from lupus, uveitis in pauciarticular childhood arthritis, all of which without intervention may result in irreversible loss of vision.  Changes in vision should be reported immediately to a physician because early intervention may be sight saving.  
      This article is reprinted  from the Lupus Courier, with permission from the Lupus Society of Alberta.

3 comments:

  1. Hi there! glad to drop by your page and found these very interesting and informative stuff. Thanks for sharing, keep it up!

    - scleritis

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    1. Thank you for dropping by and reading and commenting on my blog.

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  2. Herpes merupakan salah satu jenis penyakit kulit yang di sebabkan oleh virus. Virus herpes ini menyerang saraf tepi,maka dari itu janganheran kalau rasanya sakit sekali. Selain menimbulkan sakit saat masih terdapat luka, rasa sakit juga masih akan tetap di rasakan oleh penderita walaupun luka sudah kering dan sudah sembuh.


    cara mengobati herpes

    Herpes merupakan salah satu jenis penyakit yang sangat mudah sekali untuk menular, jadi hati-hati dan jaga kontak fisik dengan penderita herpes. Herpes itu sendiri juga di bedakan menjadi beberapa macam, sesuai dengan penyebabnya yaitu herpes simplek yang ditandai dengan luka seperti melepuh dan berisi air, herpes zoster merupakan jenis herpes yang terjadi karena penyakit varisella yang kambuh lagi, herpes genital yang berada di daerah alat kelamin, herpes labialis jika herpes terdapat pada bibir.

    Cara Mengobati Herpes S
    Herpes merupakan jenis penyakit yang disebabkan oleh virus, maka dari itu antibiotik seperti amoxcilin, ampicillin tak akan mempan untuk meredakan herpes. Karena herpes merupakan penyakit yang di akibatkan oleh virus, obatnya pun yang harus untuk membunuh virus bukan antibiotik yang berguna untuk membunuh bakteri. Ada beberapa tips yang bisa anda lakukan sebagai cara mengobati herpes. Seperti apa caranya, kita lihat yuk.

    Cara Mengobati Herpes
    Beberapa jenis obat-obatan anti virus yang bisa digunakan untuk mengatasi herpes antara lein seperti asyclovir, valasiklovir, famsiklovir. Obat-obatan tersebut khusus untuk mengobatii segala jenis penyakit yang berasal dari virus. Jadi jangan selalu berasumsi semua penyakit bisa sembuh dengan antibiotik. Karena terlalu banyak mengkonsumsi antibiotik justru akan sangat merugikan tubuh karena tubuh akan resisten dengan antibiotik tersebut.

    Kulup | Kulup panjang

    Ejakulasi dini | Sunat dewasa tak perlu malu

    Chat | Klini chat

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