Overview of Overview of Episcleritis Episcleritis and Scleritis Scleritis

6/14/2012
Overview of Episcleritis
and Scleritis
Thomas F. Freddo, O.D., Ph.D.
Professor
P f
and
dF
Former Di
Director
t
School of Optometry
University of Waterloo
Conjunctivitis vs Episcleritis
 Conjunctivitis is common. Episcleritis and
Scleritis are not.
 Apply topical anesthetic. Then use a cotton
swab
b to
t move the
th conjunctival
j
ti l surface
f
while
hil
observing through slit
slit--lamp. If the dilated and
congested vessels move with the swab, its
conjunctivitis.
 Advice on holding lid open - rollroll-up awning
Episcleritis
DDX Sectorial Redness
 A relatively benign,
recurrent disease rarely
associated with
systemic disease.
 Visual acuity is normal
 Mild, occasional pain
 More often in women
than men, as is
generally true for most
autoimmune--type
autoimmune
diseases
 Two varieties: simple &
nodular
 Staph marginal
ulcer
 Limbal
phlyctenule
hl t
l
 Limbal
malignancy
 Pterygium
Simple Episcleritis
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6/14/2012
EPISCLERITIS
 Most cases are idiopathic occurring more often
in women than men and usually
y in their 30’s or
40’s.
 A preliminary workwork-up is worth considering only
if your directed case history leads to suspicion
of an underlying systemic association.
 Systemic associations found primarily through
history in 1/3 of patients. Labs are to confirm.
 No correlation between type, laterality or
recurrences and presence of associated
systemic disease
Nodular Episcleritis
Takes longer
to resolve (4-6
weeks)
Simple Episcleritis
 Often recur at 1- to 3-month
intervals.
 Episodes appear acutely
and usually last 7-10 days
 Most resolve after 2-3
weeks.
k
 Prolonged episodes may be
more common in patients
with associated systemic
conditions.
 Some patients note that
episodes are more common
in the spring or fall.
 Precipitating factor is rarely
found.
EPISCLERITIS
Differential Diagnosis
 Virtually any infectious disease can be
associated with episcleritis – CBC with
differential , looking for leukocytosis/
leukocytosis/eosinophilia
disease, and syphilis
 Bacteria, including tuberculosis,
tuberculosis, Lyme disease,
 Viruses,
Viruses including herpes
 Fungi
 Parasites
 Other more common causes –
 Atopy - rule out by history and skin appearance
 Foreign Body – rule out by history and exam
Normal tears = 7.1
 Chemicals – rule out by history, measuring tear pH and exam
 Gout
 Adult Rheumatoid Arthritis
 Seronegative spondyloparthropathies
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If the patient is unresponsive to treatment a referral
to an cornea/external disease specialist is prudent in
order to explore the more rare causes including:
 Other rare causes/associations
 Systemic Lupus erythematosus
 Polyarteritis nodosa
 T-cell leukemia
 Paraproteinemia
 Paraneoplastic syndromes - Sweet syndrome, dermatomyositis
 Wiskott
Wiskott--Aldrich syndrome
 Adrenal cortical insufficiency
 Necrobiotic xanthogranuloma
 Progressive hemifacial atrophy
 Following transscleral fixation of posterior chamber intraocular
lens
 Insect bite granuloma
 Malpositioned Jones tube
EPISCLERITIS
EPISCLERITIS
Differential Diagnosis
Gout
Adult Rheumatoid arthritis
Seronegative spondyloarthropathies
Differential Diagnosis
Differential
Diagnosis
 Gout
Gout–
– acute attacks of foot or lower joint pain especially in great toe but
any synovial joint can be involved. Elevated uric acid level as a result of a
defect in purine metabolism. Results in deposition of crystallized uric acid
as “tophi
“tophi
tophi””. R/O in history or exam.
exam Get urine sample for uric acid level.
level In
acute attacks white count elevated with left shift and sed rate (ESR) may be
elevated. Diet. Medication of choice allopurinol
allopurinol..
 AUTOIMMUNE DISEASES
 Adult rheumatoid arthritis
 Seronegative spondyloarthropathies
 Ankylosing spondylitis
 Inflammatory bowel disease
 Psoriatic arthritis
 Reactive arthritis (Reiter’s syndrome)
Removal of tophi
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6/14/2012
Episcleritis/Scleritis and relationship with the
seronegative spondyloarthropathies
 Read and review:
www.arc.org.uk/about_arth/med_report
s/series4/tr/6604/6604.htm
s/series4/tr/6604/6604 htm
www.orthop.washington.edu/uw/tabID_
3376/print_full/ItemID_130/mid_0/Arti
cles/Default.aspx
Episcleritis – Work-up
Useful laboratory studies in this
group of patients include:
 serum uric acid - Gout
 complete blood count with
differential
 rheumatoid factor
 erythrocyte sedimentation rate
 Venereal Disease Research
Laboratory (VDRL) test
 fluorescent treponemal antibody
absorption (FTA-ABS)
 chest x-ray
If the patient is unresponsive to treatment a referral to an
cornea/external disease specialist is prudent in order to
explore the more rare causes including:
 Other rare causes/associations
 Systemic Lupus erythematosus
 Polyarteritis nodosa
 T-cell leukemia
 Paraproteinemia
 Paraneoplastic syndromes - Sweet syndrome, dermatomyositis
 Wiskott
Wiskott--Aldrich syndrome
 Adrenal cortical insufficiency
 Necrobiotic xanthogranuloma
 Progressive hemifacial atrophy
 Following transscleral fixation of posterior chamber intraocular lens
 Insect bite granuloma
 Malpositioned Jones tube
Treatment
 Ocular therapy
 Simple episcleritis often requires no treatment. Cold compresses and iced
artificial tears are useful for patients with mild-to-moderate symptoms. Patients
with severe or prolonged episodes may require topical corticosteroids but
steroids have been shown to worsen recurrences.
 Nodular episcleritis is more indolent and may require local corticosteroid drops
or anti-inflammatory agents.
 Topical pred phosphate penetrates the eye less well and thus may be preferred
over acetate in these cases to mitigate against steroid response.
 Systemic therapy
 If nodular episcleritis is unresponsive to topical therapy, systemic antiinflammatory agents may be useful.
 Flurbiprofen (100 mg tid) is usually effective until inflammation is suppressed.
 If there is no response to flurbiprofen, indomethacin should be used; 100 mg
daily and decreased to 75 mg when there is a response.
 Many patients who do not respond to one nonsteroidal anti-inflammatory agent
(NSAID) may respond to another NSAID.
 Activity
 Sunglasses may be useful for patients with sensitivity to light.
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6/14/2012
Episcleritis vs. Scleritis
Pain in the Differential
 View patient in
daylight and/or redfree.
 Episcleritis
p
will look
red, scleritis will
have a deeper,
purple-red
coloration not well
seen by slit-lamp.
 MOST Important - judge
the pain. If it is deep
penetrating pain,
radiating all around the
head and/or awakens the
patient from sleep, think
scleritis. OTC analgesics
will not lessen pain.
 If pain worsens with eye
movement, think scleritis.
DDx: Severe ocular
pain:
Corneal surface
problems
Angle closure
glaucoma
Scleritis, especially
with anterior uveitis.
Note deep purple-red background
EPISCLERITIS VS SCLERITIS:
Use 2.5% phenylephrine
phenylephrine.. Generally, it will whiten
an episcleritis
episcleritis,, not a deep scleritis
Episcleritis
Scleritis
Scleritis
 Most common
4th--6th decade
4th
 In 15% of cases,
scleritis is the
presenting sign of
collagen vascular
disease.
 More in females
1.6:1
 One
One--third are
recurrent
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6/14/2012
Scleritis
 Rheumatoid arthritis is the underlying disease for
approximately one sixth of patients suffering from scleritis
 Other connective tissue and autoimmune diseases seen with
scleritis include the following:
 Systemic lupus erythematosus (SLE)
 Polyarteritis nodosa
 Seronegative spondyloarthropathies
Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Inflammatory bowel disease
 Sarcoidosis
 Tuberculosis
 Syphilis
 Lyme
Anterior Scleritis
 Anterior
Diffuse - most
common
Nodular
Necrotizing
Most cases stay as
same type as at
original
presentation, but
some advance
*List is almost identical to anterior uveitis
Treatable Masquerades
Infectious scleritis is a possibility that should be
evaluated.
Herpes scleritis can present as a masquerade of
scleritis and should always be considered if the
patient has unilateral scleritis.
Syphilis is another important masquerader that
needs to be ruled out, as it is treatable. (Luetic
serology)
Conditions such as severe rosacea or severe
atopy or allergy can also induce scleritis, so a
thorough patient history is essential.
Diffuse Anterior Scleritis
 Most common form
 Insidious onset over 5-10
days
 Easily misdiagnosed as
episcleritis
 Increased scleral
translucency when
healed
 Least associated with
systemic disease
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6/14/2012
Nodular Scleritis
Nodule firm
and immobile
Onset insidious
Necrotizing Anterior
Scleritis
May occur
with or
without
i fl
inflammation
ti
Necrotizing Scleritis
INFLAMMATORY
NON-INFLAMMATORY
 Most severe and
destructive form
 29% die
di within
ithi 5 yrs off
onset
 50-90% have underlying
disease, usually
rheumatoid arthritis,
Wegener’s, relapsing
polychondritis
 Scleromalacia perforans
 Painless melting
g of sclera
leaving choroid exposed
to conjunctiva
 Spontaneous perforation
rare
 Most often with
rheumatoid arthritis
Wegener’s Granulomatosis
 Usually begins with respiratory symptoms












Runny nose, with puspus-filled discharge Nosebleeds
Sinus pain and inflammation (sinusitis)
Hoarse voice
Inflammation of the gums
Ear infections
Cough
Chest pain
Coughing up blood (hemoptysis
(hemoptysis))
Shortness of breath
Wheezing
General ill feeling (malaise)
But can present with scleritis or granulomatous anterior uveitis
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6/14/2012
Work up of Wegener’s
 Anti
Anti--neutrophil cytoplasmic antibodies (ANCAs) are a
group of autoantibodies
autoantibodies,, mainly of the IgG type, against
antigens in the cytoplasm of neutrophils and monocytes.
monocytes.
 They are detected in a number of conditions associated
with systemic vasculitis
vasculitis,, so called ANCE
ANCE--associated
vasculidities,, including Wegener’s granulomatosis;
vasculidities
microscopic polyangitis and Churg–Strauss syndrome.
 ANCA were originally shown to divide into two main
classes, cc--ANCA and p
p--ANCA, based on the pattern of
staining on ethanolethanol-fixed neutrophils and the main target
antigen. ANCA titers can also be measured using ELISA
and indirect immunofluorescence.
immunofluorescence.
Posterior Scleritis
Vascular congestion with hemorrhage, reduced
vision with macular stria – but with PAIN!
ANCA
 p-ANCA, or protoplasmicstaining antineutrophil
cytoplasmic antibodies,
show a perinuclear
staining pattern
pattern.
 c-ANCA, or classical
antineutrophil cytoplasmic
antibodies, show a
diffusely granular,
cytoplasmic staining
pattern.
 It is cc-ANCA that is primarily
associated with Wegener’s
Summary of Work-up
 Patients with scleritis need to be evaluated for potential systemic
diseases. I would recommend that the scleritis work-up be done by
a rheumatologist due to the wide range of conditions.
 Laboratory studies are likely to include both serologic tests and
urinalysis.
urinalysis
 Luetic serology – FTA-Abs and VDRL/RPR
 CBC with diff
 Anti-neutrophil cytoplasmic antibody (ANCA) studies will test for
Wegener’s granulomatosis.
 Various vasculidities can affect kidney and so urine protein should
be measured.
 R/o TB with CXR and PPD
B-scan: Flattening of posterior pole, Choroidal and scleral thickening with sub-Tenon’s
fluid accumulation and “T-sign”
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Based on the past history, review of systems, and physical
examination, select appropriate diagnostic tests to confirm
or reject the following suspected associated diseases:
 Rheumatoid factor - Rheumatoid arthritis
 Antinuclear antibodies - Systemic lupus erythematosus,
erythematosus, rheumatoid arthritis,
polymyositis,, progressive systemic sclerosis, or mixed connective tissue
polymyositis
 Antineutrophil cytoplasmic antibodies (ANCA) - Wegener granulomatosis
granulomatosis,,
polyarteritis nodosa
nodosa,, or microscopic polyangiitis
 Human leukocyte antigen (HLA) typing - Ankylosing spondylitis
spondylitis,, reactive arthritis,
arthritis
psoriatic arthritis, or arthritis associated with inflammatory bowel disease
 Eosinophil count/immunoglobulin E ((IgE
IgE)) - Allergic angiitis of ChurgChurg-Strauss
syndrome or atopy
 Uric acid - Gout
 Erythrocyte sedimentation rate (ESR) - Giant cell arteritis
 Hepatitis B surface antigen ((HBsAg
HBsAg)) - Polyarteritis nodosa
 Serologies - Infectious diseases, including syphilis and Lyme disease
 Purified
Purified--protein derivative (PPD) skin test - Tuberculosis
 Anergy skin test - Sarcoidosis
 Prick test - Atopy
Treatment of Scleritis
Oral NSAIDS first line, either COX-1 or more
recent COX-2 such as Celebrex at 100-200 mg
qd. More difficult cases may require oral
steroids or immunosuppressants.
immunosuppressants If NSAID use
chronic, protection of GI system may be
required (omeprazole-Prilosec)
Treatment should be coordinated with
rheumatologist of an ophtho with specialty
training in ocular immune disease.
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