Download Chicago Cornea Association

Painless Necrotizing Scleritis
with Inflammation in
Wegener’s Granulomatosis
Divya Mutyala, M.D. Robert S. Feder, M.D.
Feinberg School of Medicine
Northwestern University
Chicago, Illinois
The authors have no financial interest in the material presented
• Purpose: To report two cases of painless necrotizing
anterior scleritis with inflammation
• Methods: Retrospective chart review
• Results:
– Necrotizing anterior scleritis with inflammation was confirmed on
external and slit lamp examinations in both patients
– Both patients repeatedly denied pain and demonstrated no
tenderness on examination despite severe inflammation
– The patients were diagnosed with Wegener’s granulomatosis after
being referred for treatment
– Both patients were successfully treated with oral steroids and
systemic immunosuppressive therapy
Case 1
• 81-year-old woman with a one-month history of redness and a
mass lesion on the bulbar surface of the right eye (OD) was referred
for evaluation. She had mild photophobia, but denied discomfort
or change in vision. Past history of chronic sinus congestion and
recurrent nosebleeds.
• Examination:
– Best-corrected visual acuity (BCVA) 20/25- OD
– External examination: an elevated, injected nodule on the
temporal bulbar surface with an avascular necrotic center
measuring 4.2 x 3.0 mm OD; no abnormal pigmentation
– Slit-lamp examination (SLE): scleral thickening surrounding the
necrotic patch; scleral thinning in the avascular area; no corneal
involvement or involvement OS
– Tension, fundus examination normal
Case 1
Necrotizing Scleritis with Inflammation
• The patient was treated with prednisone 60 mg daily. Five days
later, the area of injection was diminished and the area of
ulceration was shrinking.
• A laboratory work-up revealed a positive classic antineutrophilic
cytoplasmic autoantibody (c-ANCA) at 1:640, positive anti-nuclear
antibody (ANA) at 1:40 speckled, elevated erythrocyte
sedimentation rate (ESR) at 119, and a positive Proteinase-3 ANCA
at 234.
• Computed tomography (CT) of the sinuses revealed trace mucosal
thickening in the anterior and posterior ethmoidal air cells without
evidence of bony destruction, bony thickening, or sclerosis
osteoitis.
• A diagnosis of painless necrotizing nodular scleritis OD secondary
to WG was made. Scleritis was successfully treated with
prednisone. The patient began long-term immunosuppresive
therapy.
Case 2
• A 60-year-old woman in previously good health was referred with
slowly progressive painless redness OD. The onset of the ocular
symptoms was concurrent with a chronic sinus inflammation that
had begun one month previously. Prior treatment included oral
amoxicillin, methylprednisolone, triamcinolone nasal spray and
0.5% loteprednol etabonate eye drops.
• Sinus CT was significant for bilateral mucosal thickening of the
ethmoidal and maxillary sinuses
• On examination:
– BCVA was 20/25- OD
– The external examination showed a hyperemic right upper lid
with 2+ diffuse conjunctival injection
– SLE revealed a band of peripheral keratitis superiorly OD
– There were also several non-tender areas of scleral necrosis
with inflammation at the superior limbus
Case 2
Peripheral keratitis OD
Case 2
Scleral necrosis with thinning at least 50%
• The work-up revealed a positive c-ANCA level of 1:320 and a positive
proteinase-3 ANCA at 102 units and a presumed diagnosis of WG was
made
• Treatment was initiated with oral prednisone 80 mg daily
• Over a 3-year period, the patient was managed with tapering doses of
oral prednisone and oral methotrexate with a maximum dose of 30
mg per week. Topical prednisolone acetate 1% was used
intermittently when small peripheral corneal infiltrates would emerge
OU
• The c-ANCA ultimately normalized
• Significant scleral loss with visible choroidal pigment was noted
superiorly for several clock hours and following uncomplicated
cataract surgery visual acuity of 20/30 OD sc was retained.
Discussion
• Of the different presentations of scleritis, necrotizing anterior
scleritis with inflammation is the most severe and has the greatest
potential for visual loss. Additionally, if these patients are not
appropriately treated, the areas of inflammation may widen and
cause complications of visual loss, peripheral ulcerative keratitis,
uveitis, glaucoma, and rarely corneal perforation. The involved
sclera can heal remarkably well when inflammation ceases.
• Our two patients presented with dilatation of the deep episcleral
vascular plexus with scleral necrosis and edema. Typically, pain and
scleral tenderness would herald this presentation, yet our two
patients had no discomfort. If necrosis had damaged the anterior
ciliary nerves reducing pain perception, one might expect
discomfort earlier in the course of the disease.
• The most common systemic diseases associated with necrotizing
scleritis are rheumatoid arthritis, Wegener’s granulomatosis,
relapsing polychondritis, and systemic lupus erythematosus.
• WG is a life-threatening disorder characterized by necrotizing
granulomatous inflammation of the upper and lower respiratory
tracts, vasculitis, and glomerulonephritis. Neither of our patients
suffered any pulmonary or renal complications.
• In pursuing a workup, a positive c-ANCA level and sinus CT findings
of bony destruction, bony thickening, or mucosal thickening are
helpful in providing a diagnosis of WG without a biopsy.
Additionally, the antiproteinase-3 subset of ANCA is 85% sensitive
and is a relatively specific marker for WG.
• The radiologic, laboratory and clinical evidence in our patients
helped formulate a presumptive diagnosis of WG.
Conclusion
• Early recognition of necrotizing anterior scleritis with inflammation
is essential in reducing ocular morbidity and systemic
complications. Clinicians are cautioned to avoid making the
presence of pain and tenderness determinate factors for the
diagnosis of necrotizing anterior scleritis, as this can potentially
delay the appropriate systemic evaluation, and potentially eye
saving and life saving therapeutic intervention.