Necrotizing Scleritis 2016

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

Supervisor:

Dr. Jyotirmay Biswas

MS. FNAMS., Director of Uveitis & Ocular Pathology department,

Sankara Nethralaya, Chennai, India.

Shri Bhagwan Mahavir Vitreoretinal Services,

Sankara Nethralaya,

Chennai,

India.

2015

Medical Research Foundation Chennai 600006

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

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, 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.

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

ii

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

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

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

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

Table of contents

Data management and analysis: ................................................................... 14

Ethical Considerations: ................................................................................ 15

Budget: ........................................................................................................ 15

Time plan ................................................................................................... 16

Data Checklist form of study .................................................................... 17


Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in India .......... 19

Introduction ................................................................................................. 20

Subjects and Methods .................................................................................. 20

Results ......................................................................................................... 22

Discussion ................................................................................................... 26

References… .................................................................................................. 30

vii

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

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





ix

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


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

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

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

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

Background and Review of Literature



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

inflammatory process

[8] . Inflammatory cells, mostly T cells and

macrophages, are found on biopsy specimens [8,9] . Evidence of vasculitis

with neutrophil invasion and necrosis of the vessel wall was observed in

one histopathologic study but not in a second one [8,10] . Antibody

deposition has also been described in one study [8] . One group that divided

4


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


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 connectivetissue

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


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 nonselective

or selective cyclo-oxygenase inhibitors (COX inhibitors). Nonselective

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


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


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


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

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

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:

Sample size:

• Non probability sampling; convenience sampling technique.

• Patient with necrotizing scleritis during date of study period

will be included.

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

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:

o Age (Elderly patients (>65 years) and Younger patients (≤65

years).)

o Sex (Male and female)

o BCVA

o IOP

- Frequency variables are:

o Total no. of patients with necrotizing scleritis.

o Total no. of improved eye with medication.

- Predisposing variables of necrotizing scleritis:

o Rheumatoid arthritis.

o Systemic lupus erythematosus.

o Relapsing polychondritis.

o Spondyloarthropathies.

o Wegener granulomatosis.

o Polyarteritisnodosa,

o Giant cell arteritis.

13

Methodology

- Complications variables are::

o Ant.uveitis

o S.Discoloration

o Staphyloma

o Visual loss

o Diplopia

o Limitation of ocular movement

o Proptosis

o Vitritis

o Papilledema

o Annular choroidal folds

o Exudative 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

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

Methodology

Time plan

Activities

personnel

Aug. 2014

Sept. 2014

Oct. 2014

Nov. 2014

Dece.

2014

Jun. 2015

Feb. 2015

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

Methodology

Data Checklist form for the study

Patient

MRD:

Name:

Age: Sex: USG

Investigations: Result

Eye:

Dx:

OD OS FFA

VA:

BCVA:

IOP

OCT

Ocular

Pain

Redness:

Corneal

HRCT

edema

KPs

AC Flare

AC Cell

X-ray

Vit. Cell

Pupil

Iris: Lab :

ANA

Scleritis: Anterior Posterior RF

Diffuse

ANCA

Nodular

HLA

Fundus: OD OS IgE

Uric acid

ESR

HBsAg

mantoux test

PCR

AC tape

Vitreous tape

Others

17

Methodology

Treatment:

Complications: Present

Ant.uveitis

S.Discoloration

Staphyloma

Visual loss

Diplopia

Limitation of

ocular movement

Proptosis

Vitritis

Papilledema

Annular

choroidal folds

Exudative RD

Absent

Follow-up:

Visual Acuity of effected eye post- treatment.

Follow-up visits

Duration

P.T

1-3

weeks

4-11

weeks

12 +

weeks

Date VA BCVA Pain KPs Flare AC

cell

Vit.cell

18

Clinical Profile, Laboratory Investigation and Management of Necrotizing Scleritis in Tertiary

Care Ophthalmic Centre in India


Necrotizing Scleritis in Tertiary Care Ophthalmic Centre in

India

19



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



presentation, external examination including presence or absence

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



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



Visual Acuity

Frequency Percent Valid

Percent

Cumulative

Percent

20/20 or

Better

7 19.4 19.4 25.0

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



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



(A)

(B)

Fig2 (Case 2):



(A)

(B)

Fig3 (Case 3):



25



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.


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



most common systemic condition associated with scleritis. The

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



Assessing the severity of necrotizing scleritis at an early stage is

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



to our center are difficult to control, unusual in nature. India, being a

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

References

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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

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5. Lyne AJ, Pitkeathley DA. Episcleritis and scleritis. Arch

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necrotisingscleritis. Br J Ophthalmol 2008;92:417–9.

30

References

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CS. Immunopathology of scleritis.Ophthalmology. 1991;

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9. Bernauer W, Buchi ER, Daicker B. Immunopathological findings in

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10. Bernauer W, Watson PG, Daicker B, Lightman S. Cells

perpetuating the inflammatory response in scleritis. Br J

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