Lecture: Scleral and Corneal Inflammatory Disorders

Inflammatory anterior segment disorders such as scleritis, episcleritis and peripheral ulcerative keratitis are commonly encountered in a cornea & anterior segment practice. They may have a prolonged clinical course with periodic exacerbations. These conditions may usually be associated with significant and sometimes life threatening underlying systemic diseases. The ocular manifestations are often the first or only early manifestations of such systemic conditions. Therefore, the ophthalmologist is uniquely positioned to correlate and detect these conditions at an early stage of their natural history. Treatment of these conditions requires an understanding of the underlying pathology and a multi-disciplinary approach involving the rheumatologist/internal medicine specialist. We aim to share our experience in diagnosing and managing such conditions using a case-based approach.

Lecturer: Dr. Aravind Roy, L V Prasad Eye Institute, India


DR ROY: Hello, everybody. So let me introduce myself. I am Aravind Roy. I’m a corneal consultant at L V Prasad Eye Institute in India, and today we’re going to talk about scleral and corneal inflammatory disorders. Now, this talk will be about the inflammatory conditions that we commonly encounter in anterior segment practice. So before we start the talk, could you please indicate your position? Thank you very much. So we have a lot of ophthalmologists in practice. And I’m sure in your practice you will be seeing a lot of these conditions, which you need to manage in your day-to-day practice. So how would you rate your access to rheumatology support services for your practice? Do you have these services? Or they are somewhat limited? Or you do immunosuppressants yourself? You administer them under your own care in your institution or in your practice area? Okay, that’s good to know. That there is a fair access to rheumatology. And this is very important in the management of these conditions. As we will be discussing during the course of our lecture. So why this disease is important is that it is a serious and vision threatening inflammatory disease of the eye. The reported incidence is 3.4 per 100,000 person-years. There is often an associated systemic disease, in 40% to 57% of cases, and whenever there is a serious systemic disease that is associated with this condition, the mortality rate shoots up. So some studies have reported the mortality rate from 27% to as high as 45% in three years’ duration from detection. So the minor form of the disease is episcleritis, which can be a diffuse or nodular form. The association with systemic disease does exist, and often there is a transient mild pain or ocular discomfort. One may see a nodular or a diffuse elevated mass that blanches, typically, with phenylephrine. The very transient and mild nature of this condition is often the hallmark of this condition. So there may be several autoimmune diseases that can be associated with this. It could also be associated with bisphosphonate drug reactions, or there may be miscellaneous causes, such as atopy, et cetera, that could be associated with this condition. It is a very transient, self-limiting condition, and often the first line of treatment is nonsteroidal antiinflammatory drugs and lubricants. In persistent, recurrent, or bilateral cases, which are non-responsive to conventional treatment, one can provide a short course of topical corticosteroids. So what is the least commonly associated symptom with episcleritis? Okay. So all of this can be associated with episcleritis. However, typically peripheral ulcerative keratitis does not occur or is very rarely associated with episcleritis, though can present with decreased vision. There may be a transient uveitis. There may be systemic associations, as we discussed before. However, peripheral ulcerative keratitis is often more associated with the severe form of the disease, which is scleritis. This is a patient who had recurrent attacks of redness over the past four to five years. The Mantoux was strongly positive and the ESR was raised. Other labs were negative in her case. At presentation, she presented with a triangular patch of reddishness. And very typically, as you see, the moment 10% phenylephrine was instilled in the eye, blanching was seen. So the transient recurrent nature of this condition, the typical blanching, and we can clearly see there is a superficial swelling which is very congested, consisting of dilated blood vessels, which immediately decreases in its color, and it almost blanches out to appear normal. So unless you have actually not seen the patient prior to that, one might also miss these very subtle findings in the clinical picture, as a presenting symptom of these patients. So this patient was again managed with lubricants and oral NSAIDs, as the first line therapy, and then she was referred to the physician for a Koch’s consult. That came out to be negative, and the patient was managing well with the lubricants subsequently. The more severe form of this disease is scleritis. Scleritis, again, can be divided or classified based on the anatomical sites, into anterior or posterior. Anterior scleritis can be of three different types. Diffuse, nodular, or necrotizing. The necrotizing form with inflammation or without inflammation are the two subtypes of the necrotizing anterior scleritis. Those without inflammation is also called scleromalacia. We have discussed the subtle signs, the blanching. If you have to look at a scleral nodule, and you are confused as to whether it is episcleritis or scleritis, what is the best way to look at it? So the best way to look at a scleral nodule is by natural light. The scleral nodule typically is a violaceous, reddish, bluish-red raised nodule. And often this hue is best detected under natural light conditions. So take a patient close to a window, and just examine under diffuse natural light the hue of the nodule, and often this is a very typical clinical feature, which one cannot often miss. And I’m going to show these pictures. So this is how the cases of scleritis will present. Typically, a deep boring nocturnal pain. The pain which wakes up the patient at night is something that is very classic of scleritis. There is a violaceous, rather a reddish-blue hue, congestion of deep scleral tissues, and the light focused on the nodules are very typical of scleritis. Often when you examine in free light or in green light, you can often see a capillary dropout, and often there is congestion in the deep episcleral vessels. Often there will be cases of recurrences where there will be recurrent attacks of scleritis which may be in different areas of the sclera, and once this episode passes away, with time there will be an underlying thinning of the sclera, causing uveal show, as you can see in the picture here. These are some very telltale signs of scleritis, and it may be associated with several systemic disorders, which makes it much more ominous. Scleritis can also be associated with peripheral ulcerative keratitis. So this is a very common association that you see here. Now, when there is an associated peripheral ulcerative keratitis, there are increased chances of association with systemic disease. A study found that if there is scleritis alone, the chance of an association with rheumatoid arthritis increases to 11%. However, with peripheral ulcerative keratitis, the chances increase from 38.3 to 29.2 in association with what is formerly known as Wegener’s disease. So this patient was diagnosed with scleritis. All the labs were negative. She was on azathioprine and oral prednisolone in tapering doses. This is a condition in the deep episcleral vessels. The learning point from this case is that the initial investigations, if you have to do investigation of scleritis, would be focused on RA factor and the ANCA factors. Mantoux may be needed, and this can be taken up during later visits or before starting immunosuppressants. And in areas where there is a lot of endemicity, these need to be interpreted with caution. Another case was a 41-year-old female, who presented with right eye watering, pain, and redness for three months. The left eye was essentially normal. The right eye had a diffuse condition. That was the diagnosis of diffuse scleritis, and she was both ANCA and RA positive. She was advised a rheumatologist consult, and was started on oral azathioprine, 50 milligrams. IVMP was also given. The rheumatologist planned her for four cycles of cyclophosphamide, however the hemoglobin was low. This is something that is very typical. When we manage these cases, there might be a lot of mismatch between the treatment plan and what really happens. So number one is that there might be systemic abnormalities of the patient herself, which does not allow the treatment plan to be carried forward. Number two, these patients, more often than not, present with eye complications, ocular manifestations, and it is very difficult to explain to these patients: Why do we need to do so many systemic examinations? Why do we need to send them to the internist? Why do we need to give infusions? So these are some of the real life challenges that we face when we treat this set of clinical conditions that very often come into our clinics. So this patient again proceeded with episodic attacks of redness. Diffuse congestion. Severe pain. And in the mean time, she had also intermittently some other complications. The cyclophosphamide could not be given because the hemoglobin was too low, and until that time, she was maintained on conservative management, using topical medications. We gave her only lubricants and oral azathioprine alone. So with two or three recurrences until her hemoglobin — this was a case where the disease relapsed, and then after that it went into remission. Following which, as you can see, as the congestion faded away, there is some amount of scleral thinning and uveal show. So the lab investigations in this were the CBC, hemoglobin, immune function tests, and subsequently she was shifted to oral medication. With this, she is in remission and she is maintaining in remission so far, and we are also trying to go for the improvement of her hemoglobin levels, and also, in case there is a recurrence, we now have an option to go in for the plan advised by the rheumatologist. So when we talk about scleritis, we need to understand that we need to observe these lesions, understand the presentation, and also see how these lesions look like, look for capillary dropouts, look for the violaceous hue, important to elevate the eyelid, examine the sclera for nodules, and also look for areas that may have areas of scleral thinning and uveal show. It’s important to also examine them in the free light, to look for areas of capillary dropout. The baseline investigations include the complete blood count, liver function test, renal function test, Mantoux, RBS, et cetera, before starting immunosuppressants. Mantoux positivity also needs to be taken with a pinch of salt, because in areas with a high endemicity does not always point to a Koch’s focus. Whereas, in the course of the disease, the Koch’s focus could be very ominous. So all this needs to be taken into consideration when treating scleritis and also when treating with immunosuppressants. So what are the lab investigations we should do? We should do it when there is persistent or recurrent episcleritis, all scleritis versus bilateral scleritis, unilateral necrotizing, and in cases where we suspect infection. Okay. So when we talk about laboratory investigations, it’s also important to understand that all scleritis may have associated systemic disorders. So all scleritis probably needs to be investigated. Diffuse anterior scleritis and unilateral scleritis are very common. Necrotizing scleritis may have seropositivity for ANCA, ANA, and RA. The interpretation, again, depends upon the raised ESR, C-reactive protein, serum ANCA levels, and other tests such as the CCCP. HLAs, Mantoux, infection, et cetera — these all need to be taken into account. During the management of scleritis, it’s important to look into baseline investigations. These include blood markers, Mantoux, complete blood count, and blood sugar levels. For etiological purposes, it’s important to have a baseline bouquet of tests. If you have to do tests, go ahead and do ANCA and RA factors, followed by Ds DNA, et cetera. For investigations, go for anterior fluorescein angiography. UBM, B scan, scrapings, impression biopsy, et cetera. Often there is a pattern in which one needs to approach these cases. The first line of treatment always is oral NSAIDs. Oral NSAIDs could be something like oral indomethacin or naproxen or ibuprofen. And if there is a therapeutic failure, one can switch to systemic steroids. Systemic steroids, however, after there is remission, one needs to maintain on NSAIDs. Because there are systemic issues, as well as other compliance and therapeutic failure with prolonged use of systemic steroids. So the third line of treatment is immunosuppressives, which include methotrexate, cyclophosphamide, cyclosporine, or in very resistant cases, biologics, such as infliximab. So the first study… The group presented their findings of the treatment with different types of scleritis with NSAIDs, systemic antiinflammatory drugs, which are the steroid antiinflammatory drugs, and the immunosuppressants. So this table classifies the patients who were subjected to initial treatment in different classes of drugs. So the different classes of drugs, as you can see, NSAIDs are more focused on the diffused, nodular forms of the disease, and in the necrotizing forms, the immunosuppressants are what would be advised. And this study found interesting results. As expected, the necrotizing disorders have almost 100% treatment failure with NSAIDs, which barely better performance with steroids and immunosuppressants. As you can see on the bottom right part of the table. So when they analyzed the response to drugs, the treatment failure was in the more severe form of the disease, where immunosuppressants were initially advised, and there were five treatment failures, as compared to steroids, where the initial treatment — and then they had shifted to systemic immunosuppressants. This was an interesting review of the literature on the causes and treatment of scleritis. So the data is that of systemic immunosuppressive therapy study, and the data was derived from several retrospective series. There were several studies conducted as part of these trials, and all the immunosuppressants that were compared with regards to the efficacy, meaning: What is the success when using these immunosuppressants, in order to provide a systemic disease-free remission at six months and at twelve months to patients who were recruited in the study? So when they compared methotrexate to azathioprine, mycophenolate, cyclosporine, and cyclophosphamide, the treatment success within six months was very good for those patients who had been on methotrexate and cyclosporine as compared to the other drugs. The efficacy was found to be moderately successful, and similar patterns were seen both at 6 months and at 12 months. If you can see the 12 month data of treatment success, 25% of these patients were on no systemic steroids when continued on cyclosporine, and 17.5% of patients were on no systemic steroids at 12 months when on methotrexate. The common complications of systemic immunosuppressants include: GI upset, hepatotoxicity, and systemic immunosuppression, which makes them susceptible to infections. So this was a case of a 53-year-old female who had recurrent attacks of redness in her left eye for four years. Her antinuclear antibody was found to be positive, and anti-SM antibody was also positive. She was diagnosed with lupus, which presents with non-specific symptoms. So this was the clinical picture at presentation. She was managed with systemic immunosuppressants, which led to remission. She was on oral steroids, IV cyclophosphamide, oral HCG, and azathioprine. She had episodes of remission and relapse. Often either due to the disease or due to the drugs that are administered, these patients can have a concomitant cataract. So this was a 65-year-old lady who had a 2 month history of redness, labs negative, and this was the presentation. And at presentation, she had pterygium, and there was uveal show. The disease was not very active, as you can see in the bottom pictures. So she was managed with oral methotrexate and systemic corticosteroids. These patients often have concomitant cataracts. How do we manage them? We normally look for episodes of remission. Patients are usually well managed prior to the cataract surgery with corticosteroid plus immunosuppressants. Once the disease is managed, we go for limbal or corneal phaco, and often cataract surgery has been found in our hands to be pretty safe, with no recurrence or exacerbation of the disease condition. And more often than not, these patients also do well visually, and we give them an option to be visually rehabilitated. This patient had a very good visual response, and she was 20/30 best corrected after surgery. These are her clinical pictures at day one and one month postoperative. The disease, you can see, is pretty quiet. She’s on oral steroids at a very low-maintenance dose with methotrexate. It’s often important in these conditions to make the distinction between infectious versus non-infectious scleritis. When we are dealing with an infectious etiology, you can see a very suspicious nodule in the bottom picture. These patients are usually more symptomatic. Often there might be pus pointing. Sometimes there’s a concomitant involvement of the adjacent area. Some cellularity, some infiltrate, and there might be injection. Often it is important to investigate these patients thoroughly, do a proper microbiology workup. Often it will require taking the patient to the operating room, exploring the pus-filled pockets, and sometimes it is not unusual to find additional pockets, which are not visible on the slit lamp, and on the table, when we expose the conjunctiva, there are pockets of pus which are connected to each of them. So in these cases, it is very important to completely deroof them, take the sample for microbiology, and then treat as per etiology. The other ominous finding is the scleritis that does not respond to all the conventional treatment. Are we looking at a malignancy? Yes, that’s possible. Sometimes conditions such as lymphoma or uveal coloboma can present as unusual scleritis, and one should keep masquerades as one of the differentials. With this, we come to another very interesting entity, which is called the peripheral ulcerative keratitis. That is something which is extremely common in anterior segment practice. So PUK, or peripheral ulcerative keratitis, can present with a crescent-shaped stromal inflammation. There is an overlying epithelial defect, there is progressive loss of the corneal stroma, and the adjacent conjunctiva, episclera, or sclera may also be involved. So what type of scleritis — which is most commonly seen? PUK with scleritis is most commonly seen in which condition? PUK can be associated with any of the choices that are on the screen right now. However, it can be most commonly associated with rheumatoid arthritis. There is a known association with GPA, SLE, and PAN, but by far rheumatoid arthritis is the commonest. We have a 50-year-old female patient who presented with the right eye watering, pain, and redness for 2 months. She was diagnosed as a right eye peripheral ulcerative keratitis. She had ANCA positivity, and was advised to consult rheumatology. There was a peripheral corneal furrow, and there was a suspicious area of weak sclera, that was associated with peripheral ulcerative keratitis. She was not very willing to go to the rheumatologist, so she was started in our care with oral steroids and oral azathioprine tablets. There was continued deterioration. The scleral melt continued to progress bloodlessly, and a scleral patch was applied. With repeated episodes of scleral thinning, there were areas of uveal show with thin conjunctiva covering it, and when we explored this patient on the table, we found there was a large area of the sclera which had melted out, in addition to the peripheral ulcerative keratitis. That alone could not be managed and after exposing the entire sloughed out necrotic sclera, the area of the scleral defect was measured, and the scleral patch graft was placed over this. So while this is a very simple surgery, which several of you can actually incorporate, or if you are already doing that, it’s even better, to put the scleral patch graft, however, it is important to understand that there is a scleral component, and this is the scleral patch graft, as it was integrating into the underlying sclera, posttreatment. However, it is important to understand that there are serious systemic anomalies that are associated with these conditions. Not only there is a risk of mortality, but there is also a concomitant underlying autoimmune factor. And unless these etiologies are addressed, the disease is going to be relentless. And that’s exactly what happened in this case. That in the lack of a definitive systemic treatment, the underlying disease continued to progress. So much that the scleral patch graft almost melted away. And she finally came into remission after the initiation of IV cyclophosphamide, which finally led to remission. So if we chart the clinical course, she came as a case of peripheral ulcerative keratitis, with a concomitant scleritis, that was relentless progression, requiring a scleral patch graft, and finally remission after control of the systemic condition. It’s a very similar case in a 36-year-old female, who presented with right eye redness of 5 days’ duration. The left eye was lost two years ago. We had no prior history about how this patient lost her other eye, except that there was anterior staphyloma. On examination, she had undergone right eye TA bandage contact lens 3 months earlier. The labs were weakly ANCA positive. We started her with oral corticosteroids. So this was the clinical picture of the left eye and the right eye. And she was advised a conjunctival resection and bandage contact lens for the right eye, with oral steroids and azathioprine, with which the condition resolved, over a period of three to four months. And the bottom right picture shows her current clinical picture, which is vascularized scar. The vision is hand motions. So we are planning her for penetrating keratoplasty and tarsorrhaphy. So these patients are extremely difficult to manage, and often they may require complicated surgery. The graft survival is challenging. There is always a risk of recurrence of the underlying disease process, and exacerbation of the ocular inflammation, all of which may compromise the disease process. And the graft clarity. To distinguish peripheral ulcerative keratitis from Mooren’s, we will be discussing Mooren’s in more detail after this. What is the distinction? Pain is disproportionate to the lesion. There is often overhanging edge, vascularization up to the bed of the cornea. There is often no scleritis or associated systemic disease. We can contrast this to Terrien’s, where there is superior fine yellow-white stromal opacities, with peripheral guttering, which can often be separated from the limbus by a clear zone, and there is a band of lipid at the central edge. This is a typical feature of Terrien’s, which helps us distinguish it. The causes or the associations of peripheral ulcerative keratitis can be quite wide and varied. The commonest of which would be rheumatoid arthritis and Wegener’s. The local causes would be Mooren’s or marginal keratitis, and there can be several infectious causes for peripheral ulcerative keratitis. Mooren’s ulcer is a progressive chronic ulcerative keratitis. It begins and relentlessly continues to progress throughout the cornea. There is a leading edge which is active with some cellularity. There’s an overhanging corneal edge. And the trailing edge is often thinned out and vascularized. And it continues until it involves 360 degrees of the cornea. The classic feature is that there is no concomitant involvement of the sclera. The DM is also unaffected, and often after the entire cornea has been wiped out, the disease just resolves. There is a classification based on the presentation. It could be unilateral Mooren’s, which is a painful, progressive corneal ulceration, usually seen in elderly patients. There is a non-perfusion of the superficial vascular plexus. It could be a bilateral aggressive Mooren’s ulcer, which is seen in young patients. It progresses circumferentially and then centrally. There is vascularization on the base of the ulcer. And it could be a third type, bilateral indolent Mooren’s, seen in middle aged patients, presenting with progressive peripheral corneal guttering and little inflammatory response. Often there is little change from normal vascularity. So these are the typical features in which Mooren’s can present, and it’s important to make the distinction between them and also to understand what is the underlying vascular pathology, with which it can present. More often these present with severe pain. The disease is relentless. Often they are resistant to treatment, and they continue to progress until the antigenic challenge by the corneal antigens are lost, and the cornea is replaced by thin vascularized scar tissue. There are several lab tests that one needs to do in Mooren’s. And it’s important to rule out all the known common systemic associations. So it’s very important to do the complete blood counts, ESR, RA factor, antinuclear antibody, et cetera. It’s important that etiologies such as viral or parasitic etiology has been associated with reports of Mooren’s, so it’s important to rule those out. Baseline tests are important to understand that there is no other systemic anomaly that is associated with this condition, and very often, when they start off the systemic steroids or the immunosuppressants, it’s also useful to do a conjunctival resection, with glue and BCL. This is helpful in two ways. Whenever there is an active area of Mooren’s, it’s important to do a conjunctival resection, which is done 2 millimeters from each end and 4 millimeters from the limbus. This is done so that it provides tectonic support to the thinned-out cornea. And following this, it also provides a biological barrier, by receding the conjunctiva from the lens. After that, cyanoacrylate glue is applied in a thin layer, and it can be applied to other areas which are thinned out on the cornea. So this provides a biological barrier and tectonic support, improves tectonic stability, and in addition, you can also give oral systemic immunosuppressants in managing these conditions. So early Mooren’s — there is a role of conjunctival resection. However, cases where there is extreme thinning out of the peripheral cornea, in addition to systemic immunosuppressants, one needs to do a patch graft. Or some other surgeries, which include lamellar keratoplasty, keratoepithelioplasty, tectonic grafts, et cetera. Cataract surgery is very important, because these patients after a long treatment may develop cataracts, and cataract surgery can be done, and after surgery it has been found to restore vision in patients who had various Mooren’s, with a burned out Mooren’s and vascularized scar. The importance of conjunctival resection as a primary surgery is essential. However, one needs to understand that in spite of all the best immunosuppressant and the surgical management that are options for treatment of Mooren’s ulcer, the failure rate ranges from 20% to 60%. There was a study from our group, which also published that the disease-free recurrence was 32% in the first year of treatment, and that reduced to 21% when the patients are managed only with immunosuppressants and conjunctival resection, over a period of two years. What could be the cause of decreased vision in Mooren’s? Cataracts do occur. But when there is no cataract, several factors converge to create a scenario where there is a decreased vision. What do you think is the best? As most of you have correctly answered, it could be both an iritis, an irregular astigmatism, and a corneal scar, all of which could contribute to reduced vision. So a stepladder approach for the management of Mooren’s was devised, and this was with our retrospective experience and was published from our group earlier. And this included topical steroids to be given in unilateral cases, when there was less than 2 quadrants of peripheral corneal involvement, less than 50% of stromal loss. And as the severity increased, the degree of immunosuppression increased. IVMP with IV cyclophosphamide were required in patients with bilateral, single eyed, more than 3 quadrants of peripheral corneal involvement, perforation, or in the early postoperative period after keratoplasty or patch graft. These were the hardest to manage, because they had an aggressive form of the disease, and a combined approach with methylprednisolone was used. There were 145 patients who had been treated with topical steroids alone, and there were 41 recurrences in this series, with the time to success ranging from 34 to 52 days, in the range of 1 to 521 days of follow-up. So those patients who required a little stronger version of these therapies, which included oral steroids, oral methotrexate, IVMP, IVMP with cyclophosphamide, are as shown. The overall success was very good in all of the categories, which were better than 70% in all the categories, which have been oral steroids, oral methotrexate, cyclophosphamide, and IVMP. The complications include co-infection, and there were recurrences. The recurrences occurred more with topical steroids alone, because quite obviously, this is an immune-mediated disease, and we will need a systemic immunosuppressant to manage or control this clinical condition. There was a very interesting paper that was recently published which talks about the role of rituximab in refractory Mooren’s. This was a series of five patients who were refractory to all forms of systemic immunosuppressants. The author subjected these patients to two cycles of rituximab, given in doses of 1,000 milligrams every two weeks, and they found remission in all of those cases. They concluded that in refractory Mooren’s, rituximab is a new therapeutic option and can be tried in patients where there is no other option and all the other systemic immunosuppressants have been tried. So to conclude, surface inflammations are chronic ocular disorders, they are sight threatening and can have systemic associations. Therefore they are also life threatening. More often than not, the ophthalmologist is uniquely placed in order to be the point of first contact, and it’s very important to have a team approach, where we discuss this case with the internist and the rheumatologist, and have a multidisciplinary approach to manage these cases. I would like to take this opportunity to thank my mentor and colleague and our team of fellows and optometrists who have helped us manage these difficult and challenging series of cases. Thank you very much. We’ll take a few questions before we end the session for the day.

>> Thank you, Dr. Roy. You can go ahead and stop your screen share. We have two questions right now.

DR ROY: So scleral keratitis is sometimes an infectious etiology. It could be associated with HSV, and that is both a component of the corneal and the scleral pathology, and that needs — one needs to rule out an infection. How can one identify the active end of Mooren’s ulcer? Mooren’s ulcer has a leading edge, which has a cellularity. Overhanging edge. And there is a vascularization. The trailing edge is often scarred, thinned out, and vascularized. So the difference is that the active ulcer would have epithelial defect with the overhanging edge and the cellularity. How do we decide between oral NSAIDs or steroids to start treatment in a patient with scleritis? So as we discussed, the stepladder approach to the treatment of scleritis — oral NSAIDs are the first line of management. And when there is a treatment failure with oral NSAIDs, it’s recommended to switch over to steroids. And when the disease is in remission, maintain that with oral NSAIDs. How do we preserve the sclera? I think we can take the sclera in absolute alcohol, and keep it for later use. Nevanac can be used as a topical NSAID. Cyclophosphamide can be given in a dose of 1 to 2 milligrams per kilogram, or one can give IV bolus dose in a pulse. The difference between inflamed pterygium and episcleritis is subjecting the eye to instilling one drop of 10% phenylephrine. Episcleritis tends to blanch. In recurrent PUK, can NSAID be tried first? Yes, you can try, definitely. Thyroid workup? Not routinely. We do not do that routinely. Mooren’s and Terriens. So if you recollect the picture from the lecture, Terriens would have a thinned out margin, with a clear zone between the lesion and the limbus. Often there will be vascularization and raised yellowish plaques, which are the lipids that have come out from the blood vessels. So that would have extravasated from the blood vessels. Is there any room for topical NSAIDs in sclerosis? Scleritis, I believe. I have already answered that this is the first line management, but this is with oral NSAIDs, not topical NSAIDs. I believe you can use these tissues for three months, or as long as your eye bank mentions on the accompanying papers with the tissue. The difference between Mooren’s ulcer and peripheral ulcerative keratitis is that both of them involve the peripheral cornea. However, PUK can have a concomitant episcleral or scleral involvement, whereas Mooren’s would have no scleral component, and there is no systemic involvement of Mooren’s. Mooren’s is a disease you diagnose after ruling out all the systemic possibilities. And Mooren’s can present as unilateral, bilateral, or unilateral indolent. So it can be unilateral, bilateral aggressive, unilateral indolent. Three forms. Can chemical eye injury causes scleritis? Yes, it causes that, but that is not the type of scleritis we are discussing right now. After how many days or weeks of using NSAIDs is a failure? Well, you have to review the patient on a daily or weekly basis, and if there is a worsening, then one will need to switch over to the stronger immunosuppressants, which are both oral steroids or oral immunosuppressants. Okay. So thank you for your questions. It was very exciting to interact with you. And we look forward to a few more questions before we end the talk for the day.

>> Thank you, Dr. Roy.

DR ROY: Thank you, Lawrence. I really enjoyed the session, with a lot of interaction from the participants. It really made my time.

>> Great. So since we have a few more minutes, do you mind answering some of the questions that were sent through before the talk? I can share those for you.

DR ROY: Sure. Okay. So we have two more live questions. Can we take that first?

>> Sure. Whatever you prefer.

DR ROY: So surgically induced necrotizing scleritis is something that is typically seen after an episode of surgery. More often it is after pterygium surgery, though it can present after any form of surgery. Maybe cataract or trab, et cetera. So it needs to be again managed with oral corticosteroids, as well as systemic immunosuppressants sometimes. One needs to preserve this for healing by putting a scleral patch graft, doing amniotic membrane transplant, et cetera. Okay, thank you very much. This is indeed a very challenging condition, and more often as an anterior segment surgeon, you will see several cases which will require you to have a multidisciplinary approach, and collaborate with the internist and rheumatologist, before managing these patients. The immunosuppressive drugs are given on the basis of the tolerance of the drugs and the spectrum, so it’s not about which would be more effective. Usually the second and third line drugs are given when we give oral immunosuppressants. So when we start with oral steroids, and then we also need to shift to oral immunosuppressants, and when they are refractory, shift to biologics, which would be the -mabs. So if we could go into some of the earlier questions that were there… I think the first question doesn’t really belong to the scope of the current lecture, of pediatric herpetic keratitis. However, as a cornea practitioner, I would definitely recommend that it is treated in the same way that you would deal with adult keratitis. Clinically, which way is easy to diagnose and differentiate keratopathy? That’s again beyond the scope of the current lecture. There is one live question, which is about the suture. We use the 7-0 vicryl suture for scleral patch grafts. This is the one that is used by our retina colleagues for the scleral tear repairs. How long would you use systemic immunosuppressants? We would use them, I would say, until remission and then maintain them. Effective management of acanthamoeba? Again, it is beyond the scope of the current lecture. Decide between oral NSAIDs — this we have discussed. The oral NSAIDs are the first line therapy, before shifting to a more potent form of the medication, in terms of oral steroids. The diagnosis of scleral inflammation, this is important. One needs to look for elevated nodules, diffuse involvement, violaceous hue, use natural light conditions. Often use a red-free filter or a green light to look for capillary dropouts, and also look for areas of uveal show, due to scleral thinning. There is no pediatric approach, but usually we do not see these cases in pediatric populations. Not very common. Scleral keratitis. Again, this is a concomitant involvement, where there is both a scleral and corneal involvement. More often than not, an inflammatory component with infectious background. The treatment for recurrent episcleritis — again, one needs to shift into more potent forms of immunosuppressant, and definitely look for systemic associations. Most common scleral and corneal inflammatory disease? Well, both episcleritis, scleritis, PUK, Mooren’s… We have discussed this. This is something which is very commonly seen in the anterior segment practice. But yes, the scleritis and episcleritis tend to be somewhat more common in our practice. Corticosteroid drops in keratitis? Again, I think the question is more pertaining to the role of corticosteroid in infectious keratitis, which is a very controversial topic, and this is not really pertinent to the scope of the current lecture. We have a live question, which is about the necessary labs for using immunosuppressants. As we referred to during our talk, the labs include the baseline hemogram. Look for etiology. Look for markers for any possible predisposing infection, blood sugar levels, and also look for etiological factors. After ruling them out, one needs to start with the immunosuppressants. Okay. Great. So I think we have a very productive session. And really I enjoyed the questions posed by the attendees. But I’m sure if there are further questions, you will direct them by mail to me. And we look forward to one more session with all of you too.

>> Great. Thank you, Dr. Roy. Have a great night.

DR ROY: Thank you, Lawrence. Have a great day ahead.

>> Thank you.

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March 29, 2019

Last Updated: October 31, 2022

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