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Necrotizing Scleritis 2016

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Clinical Profile, Laboratory Investigation and Management of<br>Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in<br>India

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Necrotizing Scleritis 2016

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  1. C.U. Shah Ophthalmic Post Graduate training Centre Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Presented by: Dr. Mohammad Abdullah Mohammad Bawtag M.S., Vitreoretinal Fellow, Sankara Nethralaya M.S., Vitreoretinal Fellow, Sankara Nethralaya Supervisor: Dr. Jyotirmay Biswas MS. FNAMS., Director of Uveitis & Ocular Pathology department, MS. FNAMS., Director of Uveitis & Ocular Pathology department, Sankara Nethralaya, Chennai, India. Sankara Nethralaya, Chennai, India. Shri Bhagwan Mahavir Vitreoretinal Services, Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Sankara Nethralaya, Chennai, Chennai, India. India. 2015 2015 Chennai 600006 Medical Research Foundation

  2. Dedication Dedication DEDICATION This thesis is dedicated to my mother Nabata Abdullah Zeyad Who introduced me to the joy of reading from birth, Enabling such a study to take place today. i

  3. Acknowledgments Acknowledgments ACKNOWLEDGMENTS ACKNOWLEDGMENTS First of all, and for most, all thanks to Allah, Most gracious and Most Merciful. I wish to express my deepest appreciation and profound gratitude to Prof.Dr. Jyotirmay Biswas Prof.Dr. Jyotirmay Biswas, MS. FNAMS., Director of Uveitis & Ocular Pathology department, Sankara Nethralaya, Chennai, India, for his kind support and continuous encouragement and sincere guidance throughout the preparation of this review. I would like to express my immense gratitude and appreciation to Prof. Prof. Dr. Tarun Sharma Dr. Tarun Sharma, MD, FRCSEd, MBA ,Director, Shri Bhagwan Mahavir Vitreoretinal Services , Sankara Nethralaya, Chennai, India., for his assistance, kind advice and sincere guidance throughout the preparation of this review. Many thanks to all the staff members of Ophthalmology Department and my colleagues for their continuous guidance, assistance and sincere cooperation. A special thanks to my wife who supported me throughout the writing of this thesis. Mohammad Bawtag Mohammad Bawtag ii

  4. Abstract Abstract ABSTRACT Aims: To study the Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India. Settings and Design: Methods and Material: This is a retrospective study in which we reviewed the records of 36 eyes of 31 patients of necrotizing scleritis who presented to us between August 2007 to December 2013.The study was approved by institutional review board of the Hospital. Statistical analysis used: Statistical analysis was performed Using IBM SPSS Statistics version 20 for Windows .The independent t-test was done to Obtain P value. Significance was assessed at the P≤0.05 level for all parameters. Results: Our study included 36 eyes of 31 patients of which 9 (25%) were women and 27(75%) were men. The mean age of the patients was 50.7 years (range 28- 85years). The duration of follow-up was between one month and one year. 26 patients (83.87%) had unilateral presentation to begin with while 5 patients (16.13%) developed bilateral necrotizing scleritis. The duration of symptoms and signs was variable ranging from 3days to 3 months. The most common symptoms were pain and redness 77.8% while the cases without pain and redness were 22.2%. Of all necrotizing scleritis eyes, 7 (19.4%) had some associated systemic illness. 6 were associated with rheumatoid iii

  5. Abstract Abstract arthritis, and one with Granulomatosis with polyangiitis . The remaining 29 (80.6%) cases did not have any systemic association.Laboratory studies revealed positive antinuclear antibodies in 4 cases (11.11%), positive rheumatoid factor in 6 cases (16.67%), and positive anti-neutrophil cytoplasmic antibody in 3 cases (8.33%) and raised erythrocytic sedimentation rate in 32 cases (88.89%). Conclusions: The present study details the clinical presentation, treatment, and visual outcome of Necrotizing scleritis. Systemic association is common. Aggressive therapy with systemic corticosteroid achieves resolution mostly within the first 6 months. Long-term immunosuppression is often required to prevent recurrence. Visual outcome is favourable. iv

  6. Table of contents Table of contents Table of Contents Dedication ..................................................................................................... i Acknowledgments ........................................................................................ ii Abstract ....................................................................................................... iii •Aims ...................................................................................................... iii •Settings and Design .............................................................................. iii •Methods and Material ......................................................................... iii •Statistical analysis used ....................................................................... iii •Results................................................................................................... iii •Conclusions........................................................................................... iv Table of contents .......................................................................................... v List of tables .............................................................................................. viii List of figures .............................................................................................. ix List of abbreviations .................................................................................... x Introduction ................................................................................................... 1 Justification ................................................................................................... 2 Objectives of the study: ................................................................................. 3 General objectives: ........................................................................................ 3 Specific objectives: ........................................................................................ 3 v

  7. Table of contents Table of contents Research Question ......................................................................................... 3 Background and Review of Literature .......................................................... 4 Incidence ....................................................................................................... 4 Pathophysiology ............................................................................................ 4 Risk factors .................................................................................................... 5 Clinical features ............................................................................................. 5 Complications ................................................................................................ 6 Management .................................................................................................. 7 Prognosis ..................................................................................................... 10 Methodology .................................................................................................. 11 Study design: ............................................................................................... 11 Study area: ................................................................................................... 11 Study Period : .............................................................................................. 12 Study population:......................................................................................... 12 Inclusion Criteria: ...................................................................................................... 12 Exclusion Criteria: ..................................................................................................... 12 Sampling and sample size: ........................................................................... 12 Data collection technique............................................................................. 12 Data Collection tools: .................................................................................. 13 Variables: .................................................................................................... 13 vi

  8. Table of contents Table of contents Data management and analysis: ................................................................... 14 Ethical Considerations: ................................................................................ 15 Budget: ........................................................................................................ 15 Time plan ................................................................................................... 16 Data Checklist form of study .................................................................... 17 Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India .......... 19 Introduction ................................................................................................. 20 Subjects and Methods .................................................................................. 20 Results ......................................................................................................... 22 Discussion ................................................................................................... 26 References… .................................................................................................. 30 vii

  9. List of tables List of tables List of tables Table Page Table 1: Time plan ..................................................................................... 16 Table 2: Data Checklist form for the study ................................................. 17 Table 3: Follow-up ..................................................................................... 18 Table 4: Visual Acuity ............................................................................... 23 viii

  10. List of figures List of figures List of figures Figure Page Figure 1: Case 1, (A) Necrotizing Scleritis at presentation,(B) Necrotizing Scleritis after 3 month with treatment” ...................... 24 Figure 2: Case 2, (A) Necrotizing Scleritis at presentation,(B) Necrotizing Scleritis after 3 month with treatment” ...................... 25 Figure 3: Case 3, (A) Necrotizing Scleritis at presentation,(B) Necrotizing Scleritis after 1 month with treatment” ...................... 25 ix

  11. List of abbreviations List of abbreviations List of abbreviations BCVA .......................Best corrected visual acuity CME..........................Cystoid macular edema ERM..........................Epiretinal membrane IOP ...........................Intraocular pressure RD............................ Retinal detachment VA ……………….. Visual acuity PL ………………… Perception of light NPL ………………. No perception of light NSAIDS ………….. non-steroidal anti-inflammatory drugs COX……………. …cyclo-oxygenase GI………. …………Gastrointestinal TNF…………. …….tumor necrosis factor MDR …….. ………Medical record VA………. ………..visual acuity UBM…………….. Ultrasound Biomicroscope HRCT………… ….High resolution computed tomography OCT……….. ……..Optical coherence tomography FFA………………. Fundus fluorescein angiography KPs………… …….Keratic precipitates AC………. ………Anterior chamber USG……. ………..Ultrasonography OD………. ………Right eye OS……………….. Left eye ANA…………….. Antinuclear antibody x

  12. List of abbreviations List of abbreviations RF…………. ……..Rheumatoid factor ANCA………..…..Antineutrophil cytoplasmic autoantibody HLA…………….. Human lymphocyte antigen IgE……………… Immunoglobulin E ESR……………...Erythrocyte sedimentation rate HBsAg………….. hepatitis B surface antigen PCR………………Polymerase chain reaction P.T…………. ……Post treatment xi

  13. Introduction Introduction Introduction The sclera, the dense connective tissue that encloses about five-sixths of the eye, is remarkable for its strength and firmness with which it maintains the shape of the globe. It aids in the maintenance of intraocular pressure, provides attachment sites for extraocular structures from trauma and mechanical displacement. Scleritis is a chronic, painful, and potentially blinding inflammatory disease that is characterized by edema and cellular infiltration of the scleral and episcleral tissues. Scleritis is commonly associated with systemic autoimmune disorders, including rheumatoid arthritis, systemic lupus erythematosus, relapsing polychondritis, spondyloarthropathies, Wegener granulomatosis, polyarteritis nodosa, and giant cell arteritis. Necrotizing scleritis is the most severe and destructive form of scleritis, sometimes leading to loss of the eye from multiple complications, severe pain, or perforation of the globe. The pain, always present without adequate medication, may be so intense and provoked by minimal touchn to the scalp that it may sometimes seems out of proportion to the ocular findings. Women are more likely to have scleritis than men (1.6:1). Scleritis is most common in the fourth to sixth decades of life. The peak incidence of scleritis is in the fifth decade. 1

  14. Introduction Introduction Scleritis is one ocular disorders that require urgent attention for the purpose of diagnosis, treatment, and detection of underlying systemic inflammatory diseases. The exact incidence of scleritis is uncertain, although scleritis is not common.. Previous studies have not definitively established appropriate management of Necrotizing scleritis can restore useful vision. No published evidence available on the magnitude of the necrotizing scleritis and there is no national data available to mention the magnitude of this problem in India. Justification However, the necrotizing scleritis is big problem all over the world; while, it has also Led to the increased incidence visual loss. the knowledge regarding its complication, risk factors associated with necrotizing scleritis and best management options is still evolving especially in developing countries. With an increasing number of necrotizing scleritis all over the India, estimating the need for such facilities requires registration of the incidence of this dangerous disease and its complication in the context of India. Lack of data from India reflects that the necrotizing scleritis is not well described or even investigated. This study will to some extends fill the gap in the field of knowledge concerning the necrotizing scleritis in India. 2

  15. Introduction Introduction Objectives of the study General objectives: -To study the clinical profile, laboratory investigation and management of Necrotizing Scleritis in tertiary care ophthalmic center in India. -To analyze the visual outcome, systemic associations, effectiveness of treatment Specific objectives: -To identify the factors related to occurrence of necrotizing scleritis. -To assess the relationship between the necrotizing scleritis and the factors. - To assess frequency of complications of necrotizing scleritis. -To assess the relationship between the necrotizing scleritis and systemic disease. - To assess response to medication. Research Questions: -What are the determinants of necrotizing scleritis? 3

  16. Background and Review of Literature Background and Review of Literature Background andReview of Literature Necrotizing scleritis is the most severe and destructive form of scleritis, sometimes leading to loss of the eye from multiple complications, severe pain, or perforation of the globe. The pain, always present without adequate medication, may be so intense and provoked by minimal touch in to the scalp that it may sometimes seems out of proportion to the ocular findings. Incidence: The exact incidence of scleritis is uncertain, although scleritis is not common. The reported prevalence of scleritis is skewed by the somewhat selected referrals of the reporting institutions. Scleritis is most common in the fourth to sixth decades of life. The peak incidence of scleritis is in the fifth decade. Women are more likely to have scleritis than men (1.6:1). Pathophysiology: Studies on the pathogenesis of scleritis are limited. However, the data available support an important if not predominant role for T cells in the [8]. Inflammatory cells, mostly T cells and inflammatory process macrophages, are found on biopsy specimens [8,9]. Evidence of vasculitis with neutrophil invasion and necrosis of the vessel wall was observed in [8,10]. Antibody one histopathologic study but not in a second one deposition has also been described in one study [8]. One group that divided 4

  17. Background and Review of Literature Background and Review of Literature scleritis into categories by morphology found that zonal necrotizing granulomas were more common in patients with associated systemic autoimmune conditions and that nonzonal diffuse scleral inflammation was more common in patients without an associated systemic condition [11]. Risk factors: As scleritis is associated with systemic autoimmune diseases, it is more common in women. It usually occurs in the fourth to sixth decades of life. Men are more likely to have infectious scleritis than women. Patients with a history of pterygium surgery with adjunctive mitomycin C administration or beta irradiation are at higher risk of infectious scleritis due to defects in the overlying conjunctiva from calcific plaque formation and scleral necrosis. Bilateral scleritis is more often seen in patients with rheumatic disease. Two or more surgical procedures may be associated with the onset of surgically induced scleritis. Clinical features: Symptoms of scleritis can include pain, tearing or photophobia, tenderness, and decreased visual acuity. The primary sign is redness. Pain is the most common symptom for which patients seek medical assistance, and it is the best indicator of active inflammation. Pain results from both direct stimulation and stretching of the nerve endings by the inflammation. The following pain descriptions are characteristic of scleritis: Severe, penetrating pain that radiates to the forehead, brow, jaw, or sinuses. Awakens the patient during the night. Exacerbated by touch; 5

  18. Background and Review of Literature Background and Review of Literature extremely tender. Only temporarily relieved by analgesics. Tearing or photophobia without mucopurulent discharge, which is usually mild or moderate, may occur in about 25% of patients with scleritis. Upon palpation, the patient may describe tenderness that is diffuse with possible radiation to other parts of the head. Decreased visual acuity may be caused by extension of scleritis to the adjacent structures, leading to keratitis, uveitis, glaucoma, cataract, and fundus abnormalities. Redness gradually increases over several days. It has a bluish red tinge, which is seen best when the examination is performed in natural light. It may be localized in one sector or involve the whole sclera; most frequently, it is in the interpalpebral area. This discoloration does not blanche after topical applications of routine sympathomimetic dilating agents (Neo-Synephrine 2.5%). Complications: Ocular complications of scleritis, which cause vision loss and eye destruction, appear as a result of the extending scleral inflammation. Peripheral ulcerative keratitis (13-14%), uveitis (about 42%), glaucoma (12-13%), cataract (6-17%), and fundus abnormalities (about 6.4%). These complications are most common in necrotizing scleritis, the most destructive type of scleritis. Disease association may be found in about 57% of patients with scleritis. Up to 48% of patients with scleritis present with a known connective- tissue or vasculitic disease. Some of these diseases are potentially lethal unless treated with prompt and aggressive therapy. Other patients may present with concomitant trauma, infection, or postsurgical inflammation. Systemic disease association is most common in cases of necrotizing 6

  19. Background and Review of Literature Background and Review of Literature scleritis. Scleritis may be the first manifestation of a potentially lethal systemic disease. Management: General treatment The primary goal of treatment of necrotizing scleritis is to minimize inflammation and thus reduce damage to ocular structures. Medical therapy NSAIDS Oral non-steroidal anti-inflammatory drugs (NSAI Ds) are the first-line agent for mild-to-moderate scleritis. These consist of non- selective or selective cyclo-oxygenase inhibitors (COX inhibitors). Non- selective COX-inhibitors such as flurbiprofen, indomethacin and ibuprofen may be used. Indomethacin 50mg three times a day or 600mg of ibuprofen three times a day may be used. Patients using oral NSAIDS should be warned of the side effects of gastrointestinal (GI ) side effects including gastric bleeding. Patients with renal compromise must be warned of renal toxicity . NSAIDS that are selective COX-2 inhibitors may have fewer GI side effects but may have more cardiovascular side effects. Corticosteroids Topical corticosteroids may reduce ocular inflammation but treatment is generally systemic. Corticosteroids may be used in patients unresponsive to COX-inhibitors or those with posterior or necrotizing disease. Atypical starting dose may be 1mg/kg/day of prednisone. This dose should be tapered to the best-tolerated dose. Pulsed intravenous methylprednisolone at 0.5-1g may be required initially for severe scleritis. Side effects of steroids that patients should be made aware of include elevated intraocular pressure, decreased resistance to infection, 7

  20. Background and Review of Literature Background and Review of Literature gastric irritation, osteoporosis, weight gain, hyperglycemia, and mood changes. Immunomodulatory agents I f the disease is inadequately controlled on corticosteroids, immunomodulatory therapy may be necessary . Likewise, immunomodulatory agents should be considered in those who might otherwise be on chronic steroid use. Consultation with a rheumatologist or other internist is recommended. Patients with rheumatoid arthritis may be placed on methotrexate. Patients with Wegener’s granulomatosis may require cyclosphosphamide or mycophenolate. Cyclosporine is nephrotoxic and thus may be used as adjunct therapy allowing for lower corticosteroid dosing. Mycophenolate mofetil may eliminate the need for corticosteroids. However , there is a risk of hematologic and hepatic toxicity . More recently ,tumor necrosis factor (TNF) alpha inhibitors such as infliximab have shown promise in the treatment of non-infectious scleritis refractory to other treatment. Treatment consists of repeated infusions as the treatment effect is short-lived. TNF-alpha inhibitors may also result in a drug-induced lupus-like syndrome as well as increased risk of lymphoproliferative disease. All patients on immunomodulatory therapy must be closely monitored for development of systemic complications with these medications. Medical follow up Adjustment of medications and dosages is based on the level of clinical response. Laboratory testing may be ordered regularly to follow the therapeutic levels of the medication, to monitor for systemic toxicity , or to determine treatment efficacy Surgery 8

  21. Background and Review of Literature Background and Review of Literature Clinical examination is usually sufficient for diagnosis. Formal biopsy may be performed to exclude a neoplastic or infective cause. However , one must be prepared to place a scleral reinforcement graft or other patch graft as severe thinning may result in the presentation of intraocular contents. Small corneal perforations may be treated with bandage contact lens or corneal glue until inflammation is adequately controlled, allowing for surgery. Primary indications for surgical intervention include scleral perforation or the presence of excessive scleral thinning with a high risk of rupture. High-grade astigmatism caused by staphyloma formation may also be treated. Reinforcement of the sclera may be achieved with preserved donor sclera, periosteum or fascialata. A lamellar or perforating keratoplasty may be necessary . Cataract surgery should only be performed when the scleritis has been in remission for 2-3 months. Small incision clear corneal surgery is preferred, and one must anticipate a return of inflammation in the postsurgical period. Surgical follow up Surgical biopsy of the sclera should be avoided in active disease, though if absolutely necessary , the surgeon should be prepared to bolster the affected tissue with either fresh or banked tissue (i.e., preserved pericardium, banked sclera or fascia lata). Areas with imminent scleral perforation warrant surgical intervention, though the majority of patients often have scleral thinning or staphylomaformation that do not require scleral reinforcement. 9

  22. Background and Review of Literature Background and Review of Literature Prognosis Visual loss is related to the severity of the scleritis. Patients with necrotizing scleritis have a high incidence of visual loss and an increased mortality rate. 10

  23. Methodology Methodology Methodology Study design: Retrospective case series study.., carried out on patients diagnosed as necrotizing scleritis in the Uveal departments of Ophthalmology at Sankara Nethralaya Hospital in Chennai City, India. Study area: •This study will be conducted in the non-governmental Hospital Sankara Nethralaya academy : •Sankara Nethralaya academy: Sankara Nethralaya today has grown into a super specialty institution for ophthalmic care and receives patients from all over the country and abroad. It has gained international excellence and is acclaimed for its quality care and compassion. On an average, 1200 patients walk through its doors and 100 surgeries are performed every day. Sankara Nethralaya as a High Performing Knowledge Institution – mission driven, a proven track record, acknowledged nationally and internationally for its expertise and excellence in Ophthalmic care with almost all eye sub-specialties, good management systems, a vibrant set of human resources and a participative system of decision-making. 11

  24. Methodology Methodology Study Period: Study will be donein a period from August 2014 to February 2015 Study population: This study will target all necrotizing scleritis patients undergo management in ophthalmic Uveal departments of Sankara Nethralaya in Chennai city in a period from 2009 to 2014 Inclusion Criteria: •All eyes diagnosed as necrotizing scleritis. •All age groups. •Both sexes. Exclusion Criteria: •Non necrotizing scleritis. •Necrotizing scleritis without inflammation. Sampling and sample size: •Non probability sampling; convenience sampling technique. •Patient with necrotizing scleritis during date of study period will be included. Sample size: The sample size = 33 Patient Data collection technique The data will be collected through Filling the checklist: Patient records; name, age, sex, Hospital registration number (MDR NO.). Ophthalmic examination recording of all measurements and values about present VA, BCVA or pinhole VA, then slit lamp biomicroscopy 12

  25. Methodology Methodology examination to get full details from both eyes as cornea, iris, crystalline lens ….., a pplanation tonometry to detect IOP in mmHg unit, and ultrasonography, UBM, HRCT, OCT, FFA…. The data will collected by review the patient’s files and electronic registration files. Data Collection tools: Variables: -Background variables of the patient are: oAge (Elderly patients (>65 years) and Younger patients (≤65 years).) oSex (Male and female) oBCVA oIOP -Frequency variables are: oTotal no. of patients with necrotizing scleritis. oTotal no. of improved eye with medication. -Predisposing variables of necrotizing scleritis: oRheumatoid arthritis. oSystemic lupus erythematosus. oRelapsing polychondritis. oSpondyloarthropathies. oWegener granulomatosis. oPolyarteritisnodosa, oGiant cell arteritis. 13

  26. Methodology Methodology -Complications variables are:: oAnt.uveitis oS.Discoloration oStaphyloma oVisual loss oDiplopia oLimitation of ocular movement oProptosis oVitritis oPapilledema oAnnular choroidal folds oExudative RD Data management and analysis: Data after collection will be scheduled from entry to data sheet through complex SPSS version 20 statistical software. The analysis will be through frequency and percentages for demographic variables and necrotizing scleritis. The relationships between demographic variable of patient and systemic association will be related agonist occurrence of necrotizing scleritis. The test will be used is chi squire, independent t-test and regression for associations between the variables; data will be presented in tables and graphs. 14

  27. Methodology Methodology Ethical Considerations: -Ethical approval will be obtained from Sankara nethralaya academy. -Permission from administration of the hospitals will be taken. Budget: -About≈19.500$? -Travel expenses ≈ 600 $ -Accommodation expenses ≈ 1000 $ -Food expenses ≈ 1500 $ -Telephone expenses ≈ 400 $ -Salary ≈ 6000 $ 15

  28. Methodology Methodology Time plan Sept. 2014 Oct. 2014 Nov. 2014 Aug. 2014 Feb. 2015 Jun. 2015 Dece. 2014 Activities personnel Selection of Researcher research topic Proposal Researcher Lit. Review Submission of Researcher proposal Supervisor Lit. Review Researcher Supervisors Data collection Assistants Analysis Researcher Statistician Supervisors Discussion Researcher Conclusion Supervisors Recommendations Finalizing the Researcher article Supervisors Submission of Researcher article Supervisor Discussion 16

  29. Methodology Methodology Data Checklist form for the study Investigations:Result Patient Name: Age: Eye: VA: BCVA: IOP Ocular Pain Redness: Corneal edema KPs AC Flare AC Cell Vit. Cell Pupil Iris: Scleritis: Diffuse Nodular Fundus: MRD: Sex: Dx: OS USG OD FFA OCT HRCT X-ray Lab : Anterior Posterior OD ANA RF ANCA HLA IgE Uric acid ESR HBsAg mantoux test PCR AC tape Vitreous tape Others OS 17

  30. Methodology Methodology Treatment: Complications:Present Ant.uveitis S.Discoloration Staphyloma Visual loss Diplopia Limitation of ocular movement Proptosis Vitritis Papilledema Annular choroidal folds Exudative RD Follow-up: Visual Acuity of effected eye post- treatment. Follow-up visits Duration P.T 1-3 weeks 4-11 weeks 12+ weeks Absent Date VA BCVA Pain KPs Flare AC Vit.cell cell 18

  31. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India 19

  32. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Introduction Necrotising scleritis is a rare, chronic, severe destructive inflammatory disease of the sclera. It can cause serious threat to vision and the integrity of the eye if not treated properly and timely. Management of necrotising scleritis remains a challenge to the ophthalmologist. Because of their strong association with systemic rheumatic diseases, some of which can be lethal in nature, rapid and effective management of these cases are very important. Subjects and Methods This is a retrospective study in which we reviewed the records of 36 eyes of 31patients of necrotizing scleritis who presented to us between August 2007 to December 2013.The study was approved by institutional review board of the Hospital. Necrotising scleritis was diagnosed clinically on the basis of characteristic ophthalmic findings like scleral oedema and congestion, evidence of scleral necrosis, scleral thinning. The cases with infective aetiology were excluded from the study. The hospital records were reviewed in these 36 eyes of 31 patients to determine the age, sex and history of systemic diseases. The information regarding the presenting complaints of the patient, the visual acuity and the best corrected visual acuity at time of 20

  33. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India presentation, external examination including presence or absence Care Ophthalmic Centre in India anterior segment inflammation, intraocular pressure, fundus findings were noted. Laboratory investigations, including routine haemogram, erythrocyte sedimentation rate, serum rheumatoid factor, antinuclear antibodies, and antinuclear cytoplasmic antibodies were done. Diminution of vision was defined as defined as decrease in visual acuity of 2 Snellen lines or more after resolution of the scleral inflammation clinically. Patients were treated with oral prednisolone initially (1mg/kg of bodyweight/day). Immunosuppressive were added in cases, not responding to the oral steroid, laboratory and clinical evidences of systemic rheumatic diseases as suggested by rheumatologists and in cases with recurrences. Additional treatment in the form of intravenous methyl prednisolone or cyclophosphamide was administered in patients with progressive scleral thinning in spite of the treatment with oral corticosteroids and oral immunosuppressive. Resolution was defined as a subjective improvement of best corrected visual acuity as well as the absence of any symptoms and signs of scleral inflammation on objective evaluation. The drugs were tapered and discontinued over the first 6 to12th weeks of therapy depending on the response to treatment. 21

  34. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Results Our study included 36 eyes of 31 patients of which 9 (25%) were women and 27(75%) were men. The mean age of the patients was 50.7 years (range 28- 85years). The duration of follow-up was between one month and one year. 26 patients (83.87%) had unilateral presentation to begin with while 5 patients (16.13%) developed bilateral necrotizing scleritis. The duration of symptoms and signs was variable ranging from 3days to 3 months. The most common symptoms were pain and redness 77.8% while the cases without pain and redness were 22.2%. Of all necrotizing scleritis eyes, 7 (19.4%) had some associated systemic illness. 6 were associated with rheumatoid arthritis, and one with Granulomatosis with polyangiitis . The remaining 29 (80.6%) cases did not have any systemic association.Laboratory studies revealed positive antinuclear antibodies in 4 cases (11.11%), positive rheumatoid factor in 6 cases (16.67%), and positive anti-neutrophil cytoplasmic antibody in 3 cases (8.33%) and raised erythrocytic sedimentation rate in 32 cases (88.89%). Visual acuity at presentation as showed in the following table: 22

  35. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Visual Acuity Frequency Percent Valid Cumulative Percent Percent 20/20 or 7 19.4 19.4 25.0 Better 20/30 5 13.9 13.9 38.9 20/40 2 5.6 5.6 44.4 20/60 7 19.4 19.4 63.9 20/80 3 8.3 8.3 72.2 20/120 2 5.6 5.6 77.8 20/200 2 5.6 5.6 83.3 20/400 3 8.3 8.3 91.7 CF At 50 cm 2 5.6 5.6 5.6 PL 2 5.6 5.6 97.2 NOPL 1 2.8 2.8 100.0 Total 36 100.0 100.0 There was a significant improvement in the final visual acuity when compared to presenting visual acuity. (p=0.001). Vision improved or remained stable in all patients except in 5 cases. 3 cases had complicated cataract and the other 2 cases complicated by perforation of globes during the course of the treatment. No significant observations about rise of IOP. Total number of steroid responders 34 cases (94.4%). 23

  36. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Ocular pain and tenderness was seen in 28 cases (77.8%).11 cases (30.6%) showed anterior uveitis. Most of the patient responded well to therapy with oral corticosteroid. Additional immunosuppressives were required in 8 cases. In 4 cases, intravenous methyl prednisolone was administered due to progressive scleral thinning and persistent avascularity of the scleral tissue with ongoing inflammations . One patient of Granulomatosis with polyangiitis received intravenous cyclophosphamide as advocated by the rheumatologist for systemic ailments, the treatment was continued up to 6 months. At the final follow up, 25 cases resolved completely, 9 cases were lost to follow up. Two serious complications were recorded as globe perforation in 2 eyes. (A) (B) Fig1 (Case 1): (A) Necrotizing Scleritis at presentation (B) Necrotizing Scleritis after 3 month with treatment 24

  37. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India (A) (B) Fig2 (Case 2): (A) Necrotizing Scleritis at presentation (B) Necrotizing Scleritis after 3 month with treatment (A) (B) Fig3 (Case 3): (A) Necrotizing Scleritis at presentation (B) Necrotizing Scleritis after 1 month with treatment 25

  38. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Care Ophthalmic Centre in India Discussion Scleritis is a severe inflammatory condition that is characterized by edema and inflammatory cell infiltration of the sclera. The most common presenting symptoms are the pain and redness. It is most common in the fourth to sixth decades of life, with a peak incidence in the fifth decade [1]. In 16.13% of the cases in our study it is bilateral and 83.87% unilateral. Most of the studies on scleral inflammations, showed 37- 40 % bilateral necrotising scleritis cases [2] . Being a tertiary care hospital, majority of our patients were referred from all over the country which can cause bias because of the severity of the referral cases and thus may not represent the whole disease spectrum in Indian population. This one of the reason for relatively less number of bilateral cases in our study. Necrotizing scleritis is the most severe and destructive form of scleritis, sometimes leading to severe decrese in vision from multiple complications like corneal involvements, secondary ocular hypertension, anterior uveitis , perforation of the globe etc. In our study 13.9 % of the patient developed complication during their course of scleral inflammation. Most common among them was complicated cataract. Three cases (8.33%) developed cataract and two cases (5.55%) developed globe perforation in spite best possible immunosuppression. To be able to detect the underlying disease, for proper treatment and assessment of prognosis, some diagnostic work-up is always indicated in patients with necrotizing scleritis. Rheumatoid arthritis is by far the 26

  39. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India most common systemic condition associated with scleritis. The Care Ophthalmic Centre in India reported incidence of rheumatoid arthritis in patients with scleritis is 10 to 33 % [3,4,5]. The other associated diseases are Granulomatosis with polyangiitis , systemic lupus erythematosus, juvenile rheumatoid arthritis, polyarteritis nodosa, relapsing polychondritis, psoriasis, gout, atopy, rosacea. In our study 20.83% had decreased visual acuity more than 2 lines on the Snellen chart and 79.17% gained visual acuity or lost fewer than 2 lines on the Snellen chart. Most patients with a loss of visual acuity in this study had complicated cataract or perforation. Two cases had a final visual acuity of no light perception and 3 cases lost more than 2 lines of Snellen visual acuity. In contrast to some other studies 22.1% lost more than 2 lines of visual acuity on the Snellen chart and 77.9% who gained visual acuity or lost fewer than 2 lines on the Snellen chart.[6] Although these findings regarding visual acuity in a retrospective study, [4,5] they are in accord with findings reported in the literature that loss of visual acuity is found in 15.9% to 37% of patients with scleritis. [7] Compared to other forms of scleral inflammations, necrotizing scleritis is associated with higher incidence of systemic disease. In our study 7 cases (19.4%) whith systemic disease already had been diagnosed after necrotizing scleritis onset. The most common systemic rheumatic disease was rheumatoid arthritis (16.7%), followed by granulomatosis with polyangiitis (2.8%). This difference in the number of systemic association can be explained by the relatively less number of cases in our study. 27

  40. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India Assessing the severity of necrotizing scleritis at an early stage is Care Ophthalmic Centre in India important for an adequate choice of treatment regimen. Within this study, necrotizing scleritis with; anterior uveitis and systemic disease at any time indicated a worse prognosis. In contrast to other study male gender, a longer duration of symptoms at presentation, systemic disease, and bilateral disease at any time indicated a worse prognosis. Patients in this study were treated with prednisolone 1 mg/kg/day initially. Immunosuppressives were added in cases, unresponsive to oral corticosteroid therapy, evidence of systemic rheumatic diseases as advocated by rheumatologist However majority ( 77.8%) of the cases required oral immunosuppressive medications in combination with oral corticosteroid. Most common immunosuppressive used were cyclophosphamide (63.9%), followed by mycophenolate moefetil (22.2%), Methotrexate (5.6%). 11.1 % of cases required combination of two immunosuppressives. One patient of granulomatosis with polyangiitis required intravenous cyclophosphamide therapy for control of his systemic vasculitis. However his scleral inflammation was well controlled with oral cyclophosphamide. Methotrexate was proved to be a weaker immunosuppressive in control of scleral inflammation in our study, as 27.8 % of the cases required additional or alternate immunosuppressives. On the other hand, cyclophosphamide was proved to be an effective immunosuppressive in control of necrotising scleritis. 63.9% of our patients received oral cyclophosphamide Being a retrospective study and conducted in a tertiary referral center, our study has its own biases and limitations. Most of the cases referred 28

  41. Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India to our center are difficult to control, unusual in nature. India, being a Care Ophthalmic Centre in India developing nation with his vast geography, its impractical to ascertain that all cases of necrotising scleritis cases is referred to us. Other subspecialty clinics in hospital like cornea and ocular surface disorder may see such clinical entities. Thus study conducted in a subspecialty clinic of a tertiary referral center may not reflect the exact spectrum of necrotising scleritis cases in Indian population. However we believe that the present study is among very few studies conducted on necrotising scleritis patient and add significant important knowledge in such patients in Indian scenario. 29

  42. References References References 1.Watson PG. Diseases of sclera and episclera.In Tasman W, Jaeger EA (Eds): Duane’s clinical Ophthalmology, rev ed. Lippincott, Philedelphia, 1992, pp1-43. 2.McCluskey, Wakefield D. Current concepts in the management of scleritis. Australian and New Zealand Journal of Ophthalmology 1988; 16:169-176. 3.Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol 1976; 60:163-191. 4.Mc Gavin DDM, WilliamsonJ, Forrester JV, Foulds WS, Bouchanan WN, DickWC, Lee P, Mac Sween RN, Whaley.Episcleritis and scleritis: a study of their clinical manifestations and association with rheumatoid arthritis. Br J Ophthalmol 1976; 60:192. 5.Lyne AJ, Pitkeathley DA. Episcleritis and scleritis. Arch Ophthalmol 1968, 80:171. 6.Wietse G. Wieringa..etc Visual Outcome, Treatment Results, and Prognostic Factors in Patients with Scleritis. Ophthalmology 2013;120:379. 7.Usui Y, Parikh J, Goto H, Rao NA. Immunopathology of necrotisingscleritis. Br J Ophthalmol 2008;92:417–9. 30

  43. References References 8. Fong LP, Sainz de la Maza M, Rice BA, Kupferman AE, Foster CS. Immunopathology of scleritis.Ophthalmology. 1991; 98(4):472–9. [PubMed]. 9.Bernauer W, Buchi ER, Daicker B. Immunopathological findings in posterior scleritis. IntOphthalmol. 1994;18(4):229–31. [PubMed]. 10. Bernauer W, Watson PG, Daicker B, Lightman S. Cells perpetuating the inflammatory response in scleritis. Br J Ophthalmol. 1994;78(5):381–5. [PMC free article] [PubMed]. 11. Riono WP, Hidayat AA, Rao NA. Scleritis: a clinicopathologic study of 55 cases. Ophthalmology. 1999;106(7):1328–33. [PubMed]. 31

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