Download Desai, H

AAO 2015 Resident’s Day Case Report Submission
Authors:
Heema Desai, O.D. (1); Alanna Khattar, O.D. (2); Dennis Chui, O.D. (3)
(1) Bronx Lebanon Hospital Center, optometry resident
(2) Bronx Lebanon Hospital Center, optometry resident
(3) Bronx Lebanon Hospital Center, optometry attending
Title: Scleritis Associated Early Onset Peripheral Ulcerative Keratitis
Abstract: A 41-year-old female with unilateral mild scleritis presents with an early onset
peripheral ulcerative keratitis without systemic inflammation. We discuss how early
diagnosis and treatment can help discover widespread vasculitis and prevent serious
complications.
Outline:
I. Case History
a. 41 y/o Black Female
b. Complains of redness and severe pain OS for 1 week. Reports decreased vision OS as well.
c. Ocular history:
i. H/o episcleritis OS in January 2015 (6 months prior to exam)
d. Medical history:
i. Positive PPD, Negative chest x-ray, Negative Quantiferon Gold
ii. Prediabetes
iii. Iron deficiency
iv. Benign Goiter
e. No reported allergies
f. Medications:
i. Nilfuril (French NSAID)
ii. Feosol 325mg
iii. Artificial tears
iv. Pred Forte 1% ophthalmic solution
g. From West Africa
II. Pertinent findings
a. VA OD: 20/20, OS: 20/30+
b. PERRL(-)APD, EOMs are full OU
c. Anterior segment findings:
i. OD:
Cornea:
- Nasal and temporal opacity with no cellularity
- 2+ punctate epitheliopathy inferiorly
ii. OS:
Conjunctiva/Sclera:
- 1+ erythema, no tenderness.
- Punctate staining of limbal pterygium
Cornea:
- Nasal circumferential infiltrate from 7-11 with no corneal staining
- 2+ cellularity in adjacent stroma
d. Posterior segment unremarkable
e. Corneal topography: no significant thinning OD/OS
f. Laboratory findings:
i. Sjogren’s Ab: negative
ii. ESR/CRP: within normal limits
iii. HLA-B27: negative
iv. RF/CCP: negative
v. Lysozyme: within normal limits
vi. HSV, EBV: negative for acute infection, (+) exposure
vii. Lyme: negative
viii. PPD: positive, CXR: negative, Quantiferon: negative
ix. Anti-streptolysin O (ASLO): High
x. Throat culture: negative
III. Differential diagnosis
a. Peripheral ulcerative keratitis (PUK)
b. Mooren’s ulcer
c. Terrien’s marginal degeneration
d. Marginal keratitis
e. Phlyctenular ulcers
IV. Diagnosis and discussion
a. PUK is often contiguous with adjacent conjunctival, episcleral, and scleral inflammation
(1, 5).
b. Pathogenesis: unclear
i. Hypothesized that vascular architecture of the limbus is suitable for
accumulation of IgM. Classical pathway of complement system results in chemotaxis
of inflammatory cells in the peripheral cornea. These cells release
collagenases and proteases that destroy corneal stroma. Proinflammatory
cytokines such as interleukin-1 enables stromal keratocytes to produce
matrix metalloproteinase-1 and matrix metalloproteinase-2, which can
accelerate matrix destruction process (2, 5).
c. Stages of PUK:
i. Opacity due to cellular infiltrates within the stroma adjacent to the limbus, without
initial epithelial defect (5).
ii. After some time, crescent-shaped corneal ulcers can develop with
breakdown of overlying epithelium. (1)
iii. Varying degrees of vascularization and corneal thinning due to tissue loss
in the underlying stroma can occur (1,4)
d. PUK is the initial manifestation of collagen vascular disease in 50% of cases (1, 5)
i. Rheumatoid arthritis (RA) is the most commonly associated systemic disease. PUK
usually presents at the later stages of RA
ii. PUK occurs at the earlier stages in patients with Wegener granulomatosis, PUK is
often the presenting sign of Wegeners.
e. Proper diagnosis is critical in order to determine treatment. Essential to rule out:
i. Mooren’s ulcer: form of PUK, but is not associated with scleritis (5)
ii. Terrien’s marginal degeneration: inflammation and epithelial defects are not
hallmarks of Terrien’s marginal degeneration (1)
iii. Marginal keratitis: usually space is present between the ulcer and the limbus.
The signs are less severe and are usually self-limited. Will also respond rapidly to
topical treatment, whereas PUK, it might worsen (5)
iv. Phlyctenular keratitis: less severe and self-limiting.
e. Early treatment is critical to prevent corneal complications such as (3, 4):
i. Corneal perforation
ii. Anterior uveitis
iii. Decreased vision
iv. PUK is often associated with systemic morbidity, when PUK is in the setting of
systemic disease.
f. Treatment options include (5):
i. Systemic glucocorticoids (Methylprednisone)
ii. Alkylating agents (Cyclophosphamide, Chlorambucil)
iii. Antimetabolites (Methotrexate, Azathioprine)
iv. T-cell inhibitors
v. Biologic agents (Infliximab)
V. Treatment and management
a. Day 1: Pred Forte oph soln 6x/day OS (topical glucocorticoid appropriate due to lack of
epithelial defect)
b. Day 2: Bloodwork to rule out any systemic inflammation. Continue Pred Forte oph soln
6x/day
c. Day 25: After consultation due to positive PPD, patient will report to infectious disease.
Consider starting course of Isoniazid and Vitamin B6 before initiating Methotrexate for
long-term treatment
d. Punctal plugs LLL and LUL for treatment of dry eyes
e. Consult Rheumatology for monitoring and long-term immunosuppression after Infectious
Disease exam
VI. Conclusion
a. Peripheral ulcerative keratitis is a rare condition usually associated with increased ocular
morbidity and also mortality
b. Early cases of peripheral ulcerative keratitis can present as an opacity due to cellular
infiltrates within the stroma adjacent to the limbus, without ulceration.
c. Early diagnosis and treatment is critical to prevent ulceration and other corneal
complications. It can also potentially catch a more severe systemic inflammation such as
Rheumatoid arthritis, Wegener’s granulomatosis, polyarteritis nodosa and systemic lupus
erythematosus.
i. Extensive laboratory testing is important for the proper diagnosis
ii. Systemic glucocorticoids and long-term
immunosuppressive/immunomodulators are the mainstay of treatment
d. Role of optometry:
i. Good case history to indicate widespread systemic inflammation, rule out
conditions such as Tuberculosis
ii. Proper bloodwork and diagnosis
iii. Co-management as a multidisciplinary team with Internal Medicine,
Rheumatology and Infectious Disease
iv. Close follow-up to monitor and ensure resolution
References:
1. Galor, A. and Jennifer E. Thorne. “Scleritis and Peripheral Ulcerative Keratitis.”
Rhuem Dis Clin North Am. 2007 November; 33(4): 835-854.
2. Gomes B., Priscilla Almeida Jorge, et al. “Corneal Involvement in Systemic
Inflammatory Diseases.” Eye & Contact Lenses. May 2015; Volume 41, Number 3.
3. Okhravi, N., Bola Odufuwa, et al. “Scleritis.” Survey of Ophthalmology. July-August
2005. Volume 50, Number 4.
4. Sainz de la Maza M., Stephen Foster, et al. “Ocular Characteristics and Disease
Associations in Scleritis-Associated Peripheral Keratopathy.” Arch Opthalmol. 2002;
120:15-19.
5. Yagci, Ayse. “Update on Peripheral Ulcerative Keratitis.” Clinical Ophthalmology.
2012:6 747-754.