Scleritis is a serious inflammatory disease that affects the white outer coating of the eye, known as the sclera. The disease is often contracted through association with other diseases of the body, such as granulomatosis with polyangiitis or rheumatoid arthritis

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3. Scleritis: When a Red Eye Raises a Red Flag
This painful inflammation is often an indicator of more serious health
concerns.
 Scleritis refers to chronic inflammation of sclera proper.
 Usually occurs in elderly group (40-70years)involving female more than male.
 Scleritis manifests as a very painful red eye—but it sometimes suggests that something
deeper than the eye is involved. It’s often, but not always, associated with an underlying
autoimmune disorder. So, it’s vitally important to get to the bottom of this uncommon
but aggravating condition.
 Scleritis can be classified as either anterior or posterior, where anterior scleritis can be
further categorized into four different types:
 Diffuse scleritis is widespread inflammation of the sclera, and the most common type.
 Nodular scleritis is characterized by a localized area of inflammation where a distinct
nodule can be seen

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 Necrotizing scleritis with inflammation is frequently associated with collagen vascular disorders causing
destruction of the sclera.
• Necrotizing scleritis without inflammation, also known as scleromalacia perforans, is a very rare form of
scleritis presenting with no symptoms. It can be seen in patients with rheumatoid arthritis, granulomatosis with
polyangiitis (formerly known as Wegener’s granulomatosis) and relapsing polychondritis)
 Posterior scleritis can also be nodular or diffuse and necrotizing, and involves the sclera posterior to the
insertion of the rectus muscles.

5. CASE HISTORY
 A 66-year-old Indian male presented with bilateral red and mildly
painful eyes. He said his eyes had been getting red on and off, but this
episode was much worse.
PATIENT HISTORY:
 Done cataract surgery (OU) since 5 years
 Positive family ocular history

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 Systemic disease like chronic respiratory issues with a persistent cough for the past four to six months.
 Diagnosed with recurring bronchitis and was treated with several courses of antibiotics as well as oral
steroids.
 He had also lost an unexplained 20 lbs.
 Reporting generalized muscular pain.
 He was under medication at the time of visit. Durezol [difluprednate 0.05% QID]

7. DIAGNOSTIC DATA
VISUAL ACUITY
OD:20/25
OS:20/30
IOP
OD:18mmHg
OS:19mmHg
EOM: FULL OU;NO NYSTAGMUS
SLIT LAMP
showed bilateral sectoral nodular scleritis
[mostly superficial and some deeper episcleral
inflammation noted]
Fig; Sectoral nodular scleritis

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 Considering his bilateral scleral inflammation along with his systemic issues,
additional lab testing was ordered, which included: RF, ANA, HLAB27, Lyme, PPD,
FTA-ABS, ACE, C-ANCA, P-ANCA, ESR, CRP.

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ommon Laboratory Tests for Patients with Scleritis
Laboratory Test Systemic Condition
ACE (angiotensin-converting
enzyme)
Chest X-ray
Sarcoidosis
ANA (antinuclear antibody) Lupus
c-ANCA (cytoplasmic
antineutrophil cytoplasmic
antibody)
Wegener's granulomatosis
p-ANCA (perinuclear
antineutrophil cytoplasmic
antiybody)
Vasculitis, polyarteritis nodosa
FTA-ABS (fluorescent
treponemal antibody
absorption)
RPR/VDRL (rapid plasma
reagin/venereal disease
reference laboratory)
Syphilis
ELISA (enzyme-linked
immunosorbent assay)
Western blot
Lyme disease
RF (Rheumatoid factor) Rheumatoid arthritis
CRP (C-reactive protein)
ESR (erythrocyte
sedimentation rate)
Nonspecific systemic
inflammation
Common Laboratory Tests for Patients with Scleritis
Laboratory Test Systemic Condition
ACE (angiotensin-converting enzyme)
Chest X-ray
Sarcoidosis
ANA (antinuclear antibody) Lupus
c-ANCA (cytoplasmic antineutrophil cytoplasmic
antibody)
Wegener's granulomatosis
p-ANCA (perinuclear antineutrophil cytoplasmic
antiybody) Vasculitis, polyarteritis nodosa
FTA-ABS (fluorescent treponemal antibody
absorption)
RPR/VDRL (rapid plasma reagin/venereal disease
reference laboratory)
Syphilis
ELISA (enzyme-linked immunosorbent assay)
Western blot
Lyme disease
RF (Rheumatoid factor) Rheumatoid arthritis
CRP (C-reactive protein)
ESR (erythrocyte sedimentation rate)
Nonspecific systemic inflammation

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 When he returned one week later, his eyes were white and quiet. His lab results was reviewed, which
showed significantly elevated ANCA and CRP levels. His pulmonologist was contacted due to a high
concern for granulomatosis with polyangiitis (GPA, aka Wegener’s granulomatosis); imaging studies and a
lung tissue biopsy later confirmed the diagnosis. The patient was sent to a GPA specialist for further
evaluation and treatment.

11. DISCUSSION
 Half of all scleritis cases have an underlying systemic cause. So, a thorough history and review of systems
are very important to help identify any underlying etiologies and guide laboratory studies or further medical
evaluation.
GPA is often overlooked due to its rare incidence—just three cases per 100,000 people.Approximately 10%
of patients with GPA develop scleritis, so ANCA testing should be included in the workup of these patients.
 In addition to GPA, rheumatoid arthritis, relapsing polychondritis, polyarteritis nodosa, systemic lupus
erythematosus, sarcoidosis, anklyosing spondylitis herpes zoster, syphilis and tuberculosis are associated
with scleritis and should be tested as causative factors.
 The patient returned for follow-up six months later. He was tapering prednisone and had started
methotrexate for the GPA. The overall improvement in his health was remarkable. He had gained back 20
lbs., and was feeling significantly better with no more breathing or coughing issues. His scleral inflammation
has been quiet since.

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