Download Necrotizing Scleritis

OCULAR ONCOLOGY CASES FROM COLE
Section Editor: Arun D. Singh, MD
Necrotizing Scleritis
Up to half of patients with this condition have an underlying systemic disease.
BY XIANG Q. WERDICH, MD, P H D; CAREEN Y. LOWDER, MD, P H D;
AND ARUN D. SINGH, MD
CASE PRESENTATION
A 46-year-old white man was referred to our clinic for an
evaluation of a conjunctival growth of 4 month’s duration
in his left eye. He reported that, during this period, he had
experienced intermittent photophobia, redness, and
blurred vision. He had been treated with oral antibiotics
without significant improvement. Though helpful, oral
steroids also did not resolve his symptoms completely.
During the previous 3 to 4 weeks, his left eye had become
more injected with the development of a growth over the
conjunctiva. He reported that his left eye was very sore,
often keeping him awake at night. The patient also recalled
that, 2 to 3 months before the current episode, he had
experienced blurred vision and photophobia in both eyes,
which subsided spontaneously. A review of his medical history was significant for psoriasis without joint involvement,
essential tremor, and a slowly healing skin wound of the left
leg that lasted for about 6 months.
On examination, the patient’s BCVA was 20/25 in the
right eye and 20/50 in the left. The conjunctiva of both
eyes was noticeably injected, with the left eye worse than
the right, and the conjunctiva was not blanched by the
use of 2.5% topical phenylephrine. In the left eye, the
temporal conjunctiva was slightly thickened with areas of
capillary nonperfusion and scleral necrosis (Figure 1A).
There was white infiltrate of the temporal periphery of
the cornea associated with thinning and ulceration
(Figure 1B). Keratic precipitates on the corneal endothelium and 2+ cells in the anterior chamber were also
observed. In the right eye, sections of the sclera appeared
to have a blueish-violet hue. The dilated fundus examination was normal in both eyes.
The patient’s ocular findings, diagnosed as necrotizing
scleritis and peripheral ulcerative keratitis, were most likely associated with an underlying autoimmune disorder.
DISCUSSION
Scleritis Presentation
Scleritis is a severe, painful inflammatory process of the
sclera with a subacute onset that frequently involves the
overlying episclera, underlying uvea, and the cornea. It is
A
B
Figure 1. Slit-lamp images of the left eye; the pupil was dilated with 2.5% topical phenylephrine. Areas of avascular or
necrotic sclera (arrows) (A) and peripheral corneal infiltration
with thinning and ulceration (arrow) (B).
often progressively destructive and can be a significant
threat to the integrity of the globe and the patient’s
vision. Scleritis occurs most often in the fourth to sixth
decades of life, is more common in women compared
with men, and is exceedingly rare in children. Most
patients with scleritis develop severe, boring eye pain
that is exacerbated by the eye’s movement and that is
worse at night. The inflamed sclera has a bluishMAY/JUNE 2010 ADVANCED OCULAR CARE 25
OCULAR ONCOLOGY CASES FROM COLE
“A careful examination
of the patient’s other eye
often provides important clues
for diagnosing scleritis
in the affected eye.”
violaceous hue that cannot be blanched with the application of 2.5% topical phenylephrine.1 About half of scleritis cases have bilateral involvement.
A careful examination of the patient’s other eye often
provides important clues for diagnosing scleritis in the
affected eye. As in our patient, the bluish-violet hue of
the right sclera indicated that he likely had had previous
attacks of scleritis and that the left eye likely was currently in an active phase of the disease.
Etiology
The etiology of scleritis can be infectious, induced by
surgery or trauma, or related to intraocular tumors.
However, up to 50% of patients with scleritis have evidence of an underlying systemic connective tissue or
vasculitic disease.2 The most common systemic associations of scleritis include rheumatoid arthritis, Wegener’s
granulomatosis, relapsing polychondritis, systemic lupus
erythematosus, polyarteritis nodosa, and arthritis with
inflammatory bowel disease. Thus, the presence of scleritis warrants a thorough diagnostic and systemic evaluation, guided by the patient’s individual presentation.
Differentiation of scleritis from episcleritis is important,
as the latter is usually a self-limited acute condition
with rare adverse ocular sequelae and an infrequent
association with systemic diseases. Generally, it requires
no more than systemic nonsteroidal anti-inflammatory
drugs for treatment.
Compared with other forms of scleritis such as diffuse
or nodular, necrotizing scleritis is the most destructive.
Affected patients experience severe pain and present
with white avascular necrotic areas surrounded by scleral inflammation. Untreated, necrotizing scleritis may
spread posteriorly or circumferentially and result in
severe tissue loss. The sclera may develop a blue-gray
appearance due to thinning, which allows visualization
of the underlying choroidal structure. Scleral biopsy
should be avoided in these patients, as it will result in
the tissue’s not healing. As alluded to previously, necrotizing scleritis is most frequently associated with
Wegener’s granulomatosis.
This patient’s workup was significant for trace protein
levels in the urine, a high sedimentation rate (WSR 85),
26 ADVANCED OCULAR CARE MAY/JUNE 2010
and positivity for antineutrophil cytoplasmic antibody
(c-ANCA). Chest computed tomography demonstrated
several ground-glass nodules scattered in the bilateral
lung fields and a few small calcified granulomas at the
base of the left lung. Together with the clinical presentation, he was diagnosed with Wegener’s granulomatosis, a multisystem autoimmune disorder with a classic
pathological triad of small-vessel vasculitis, tissue necrosis, and granulomatous inflammation.
Therapy
Necrotizing scleritis associated with Wegener’s granulomatosis mandates combination therapy using systemic
corticosteroids, typically prednisone (1 mg/kg/day), and
immunomodulatory agents, typically cyclophosphamide
(1-3 mg/kg/day). Intravenous methylprednisolone is
occasionally used for the rapid control of the inflammatory response, such as in the setting of threatened scleral
or corneal perforation. The goal of the therapeutic regimen is not only to treat scleral inflammation but also to
control the underlying systemic vasculitis, which has a
1-year mortality rate of 80% without treatment. With
therapy, 93% of patients will successfully achieve remission.3 A close collaboration between the ophthalmologist
and the rheumatologist is crucial for the effective management of these patients.
OUTCOME
Our patient received three doses of intravenous
methylprednisolone (1 g/day) and was then switched to
oral prednisone 80 mg/day. He was also started on oral
cyclophosphamide 175 mg/day. His symptoms significantly improved within 5 days of the initiation of treatment. It is important to maintain this regimen until
total control of ocular inflammation is achieved. ■
Section editor Arun D. Singh, MD, is director
of the Department of Ophthalmic Oncology at
the Cole Eye Institute, Cleveland Clinic
Foundation. Dr. Singh may be reached at
(216) 445-9479; [email protected].
Careen Y. Lowder, MD, PhD, is a staff physician, and Xiang Q. Werdich, MD, PhD (pictured),
is a resident at the Cole Eye Institute, Cleveland
Clinic Foundation. Dr. Lowder may be reached at
(216) 444-3642; [email protected]. Dr. Werdich
may be reached at (216) 464-8410, ext. 84845;
[email protected].
1. Sainz de la Maza M,Vitale AT.Scleritis and episcleritis.In:Focal Points:Clinical Modules for Ophthalmologists.San
Francisco,CA:American Academy of Ophthalmology;2009;xxvii (4):1-14
2. Okhravi N,Odufuwa B,McCluskey P,Lightman S.Scleritis.Surv Ophthalmol.2005;50(4):351-363.
3. Kersten R.Wegener granulomatosis.In:Basic and Clinical Science Course.San Francisco,CA:American Academy of
Ophthalmology;2009;Section 9 Intraocular Inflammation and Uveitis;Chapter 7,Noninfectious (Autoimmune)
Uveitis:177-179