Dr. Sowkath Ali B, Dr. Jyotirmay Biswas
Presenting authors:
1.B. Sowkath Ali
Fellow, Department of uvea,
Sankara Nethralaya,
Chennai
2.Jyotirmay Biswas
Professor of Ophthalmology
Department of uvea
Sankara Nethralaya,
Chennai
ABSTRACT:
Aim: To describe the clinical and epidemiological profile of necrotizing anterior scleritis in a tertiary care centre.
Methods:Retrospective case series of patients with presumed diagnosis of necrotizing scleritis were enrolled by reviewing their case records over a period of 8 years.
Results: A total of 36 patients were enrolled, (males- 24, females -12)of which 32 had unilateral involvement (Right – 12, left – 20). Mean age of presentation was 46.83 years,Supero temporal quadrant was most commonly involved (n=24), history of connective tissue disorder seen in 8 patientswith C-ANCA positivity in 7 patients.Cyclophosphamide was the most used immunosuppressive (n=35), followed by Mycophenolate Mofetil (n=2). Scleral perforation was seen in 3 eyes for which grafting has been done and secondary glaucoma was seen in 4 eyes.
Conclusion: Necrotizing scleritis is a vision threatening disorder and prompt recognition and management with immune suppressiveswill prevent its complications.
INTRODUCTION:
Scleritis is a chronic, painful, and potentially blinding inflammatory disease that is characterized by edema and cellular infiltration of the scleral and episcleral tissues.1 Scleritis most often presents within 4th–6th decades with a mild preponderance towards women over men (1.6:1).2 The prevalence of scleritis in the general population is estimated to be 6 cases per 100,000 people, but has been described in between 0.2% and 6.3% of patients with RA and up to 7% of those with Wegener’s granulomatosis. 3The Watson and Hayreh classification of scleritis divides the disorder into anterior and posterior types based upon the anatomic distribution of disease and the observed alterations in the associated vascular structures. Anterior scleritis is further subdivided into diffuse, nodular, necrotizing with inflammation, and necrotizing without inflammation (scleromalacia perforans).3
In the Watson and Hayreh series, connective tissue disorders were present in 15 % ofthe patients, of which rheumatoid arthritis constituted10 %.Necrotizing scleritis has the highestassociation with systemic illness and represents themost frequent type of scleritis that is the firstmanifestation of a systemic condition.1 Surgically induced necrotizing scleritis (SINS) can occur after any type of ocular surgery with scleral manipulation.1
There is a wide range of choices for the treatment of necrotising anterior scleritis, from corticosteroids to monoclonal antibodies targeting an inciting molecule. Corticosteroids have the advantage of rapid control of inflammation, but carry many systemic risks with long-term therapy. Immunemodulatory therapy can sustain inflammatory control without the same side effects that can be caused by long-term steroid use.4
This paper highlights the clinical and epidemiological profile of necrotizing anterior scleritis in a tertiary care centre and highlights the importance of immunosuppressive therapy for the control of it.
RESEARCH METHODOLOGY:
STUDY DESIGN: Retrospective interventional non comparative case series
STUDY PERIOD: 8 years
STUDY METHODS:
All case records of clinically confirmed cases of necrotizing anterior scleritis over a period of 8 years from 2008 to 2015 were reviewed. All were examined by a single ophthalmologist. A detailed history including duration of symptoms, associated history of systemic connective tissue disorder, epidemiological profile including age, gender was noted. A complete ophthalmic evaluation including best corrected visual acuity, intra ocular pressure, slit lamp evaluation and fundus evaluation were noted. Blood investigations done including RA, ANA, c-ANCA, p-ANCA were noted. Treatment history including the duration of the treatment, type of immunosuppressive used were noted. Patients were usually kept in regular follow up for monthly every 3 months followed by every 2 months. BCVA and resolution of signs and symptoms at all visits were noted. Occurrence of complications if any was also noted.
RESULTS:
A total of 36 patients were enrolled, (males- 24, females -12) of which 32 had unilateral involvement (Right – 12, left – 20) and 4 had bilateral involvement. (Total number of eyes – 40).Mean age of presentation was 46.83 years, the most common age group being between 50 to 60 years, followed by 40 to 50 years. Redness was the most common complaint (N=36) followed by defective vision. Supero-temporal quadrant was most commonly involved (n=24), history of connective tissue disorder seen in 8 patients with C-ANCA positivity in 7 patients. Best corrected visual acuity showed significant improvement between pre and post treatment (60 % between 6/18 to 6/6 pretreatment to 78% between 6/18 to 6/6 post treatments).intra ocular pressure was normal in 33 patients at the time of presentation, elevated in 3 patients. Fundus examination was normal in 28 patients, 8 patients showed abnormal fundus findings at the time of presentation in the form of ( cupping in 3 patients, changes of diabetic retinopathy in 4 patients, status post belt buckle surgery changes in 1 eye). One patient had surgically induced necrotizing scleritis following pterygium excision and was treated with intravenous methyl prednisolone followed by cyclophosphamide. Cyclophosphamide was the most used immunosuppressive (n=35), followed by Mycophenolate Mofetil (n=2). Scleral perforation was seen in 3 eyes for which grafting has been done and secondary glaucoma was seen in 4 eyes. Mean duration of the treatment was found to be 1.45 years.
DISCUSSION:
In a retrospective review of patients within the Systemic Immunosuppressive Therapy for Eye Disease (SITE) 51 eyes from 33 scleritis patients that were treated with MMF, 25.5% had successful control of inflammation at 6 months and 49.5% at 12 months while on prednisone ≤ 10 mg. Complete therapeutic success, i.e. control of inflammation off all steroids, occurred in 7.1%.5In the SITE cohort, 76 eyes of 48 patients with scleritis were treated with cyclophosphamide, 47.9% of whom were treated with another immune suppressant first. 30.2% of patients achieved treatment success at 6 months on less than 10 mg of prednisone and 60.5% were controlled at 12 months. 15.9% were controlled at 12 months on cyclophosphamide alone.6
In our study, complete resolution of success was attained in 85% of patients treated with cyclophosphamide alone (3 eyes developed scleral perforation while on treatment).Connective tissue disorder association with concomitant c-ANCA positivity was seen in 20 % of patients, confirming Wegener’s granulomatosis which is comparable to other studies.
CONCLUSION:
Scleritis is a potentially blinding disorder and the mainstay of treatment should be the use of a steroid sparing agent, in order to achieve complete remission. Choice of immunosuppressive depends on the concomitant occurrence of connective tissue disorder, severity of inflammation and patient adherence profile. Proper initiation of immune suppressive therapy can prevent potentially blinding complications.
REFERENCES:
1.ZahedurRahman ,Jyotirmay Biswas . Current Approach in Diagnosis and Management of Scleritis – major review. Kerala Journal of Ophthalmology. 2008; 4:341-348
2.Watson PG, Hayreh SS. Scleritis and episcleritis. Br JOphthalmol 1976;60:163-91
3.Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin PractRheumatol. 2007;3(4):219–26
4.Galor, A., & Thorne, J. E. (2007). Scleritis and Peripheral Ulcerative Keratitis.Rheumatic Diseases Clinics of North America, 33(4), 835–854.
5.Sobrin L, Christen W, Foster CS. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis. Ophthalmology. 2008;115:1416–21.
6.Pujari SS, Kempen JH, Newcomb CW, et al. Cyclophosphamide for ocular inflammatory diseases. Ophthalmology. 2010;117:356–65