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Peripheral Ulcerative Keratitis
Article Last Updated: Jun 2, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Ellen N Yu, MD, Consulting Staff, Department of Ophthalmology, St Luke's Medical Center, Quezon City, Philippines
Ellen N Yu is a member of the following medical societies: American Academy of Ophthalmology and Philippine Medical Association
Coauthor(s):
C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
Editors: Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Author and Editor Disclosure
Synonyms and related keywords:
PUK, marginal corneal ulcer, corneal stroma, corneal perforation, rheumatoid arthritis, RA, vision loss, blindness
Background
Peripheral corneal ulceration is a potentially devastating disorder consisting of a crescent-shaped destructive inflammation at the margin of corneal stroma that is associated with an epithelial defect, presence of stromal inflammatory cells, and progressive stromal degradation and thinning. Commonly referred to as peripheral ulcerative keratitis (PUK), it can quickly produce progressive necrosis of the corneal stroma, leading to perforation and blindness (Mondino, 1988).
Pathophysiology
The peripheral cornea has distinct morphologic and immunologic characteristics that predispose it to inflammatory reactions. Unlike the avascular central cornea, the peripheral cornea is closer to limbal conjunctiva and derives part of its nutrient supply from the limbal capillary arcade, a source of immunocompetent cells, eg, macrophages, Langerhans cells, lymphocytes, and plasma cells (Shiuey, 1998; Messmer, 1999). Any inflammatory stimulus in the peripheral cornea that is caused by invasion of microbial organisms (bacteria, virus, fungi, and parasites), immune complex deposition (in systemic immune diseases), trauma, malignancy, or dermatologic conditions may produce local and systemic immune responses, resulting in neutrophil recruitment and complement activation (both classic and alternative pathways) in both tissue and vessels (Shiuey, 1998).
Activated complement components can increase vascular permeability and further generate chemotactic factors for neutrophils (eg, C3a, C5a). Neutrophils, in turn, infiltrate the peripheral cornea and release proteolytic and collagenolytic enzymes, reactive oxygen metabolites, and proinflammatory substances (eg, platelet-activating factor, leukotrienes, prostaglandins), causing dissolution and degradation of the corneal stroma (Foster, 1993; Gregory, 1996). In addition, the inflamed limbal conjunctiva itself is capable of producing collagenase, which contributes to stromal degradation (Eiferman, 1979).
Systemic diseases that may cause immune complex deposition at the peripheral cornea and PUK include such collagen vascular diseases as rheumatoid arthritis (RA), Wegener granulomatosis (WG), polyarteritis nodosa (PAN), relapsing polychondritis (RP), and systemic lupus erythematosus (SLE). Infectious conditions, whether systemic (eg, hepatitis, syphilis) or local (eg, herpes simplex keratitis, fungal keratitis), and noninfectious local disorders (eg, Mooren ulcer, marginal keratitis) also may cause PUK.
In summary, the major pathophysiologic mechanism of PUK is a result of degradation and tissue necrosis of corneal stroma produced by degradative enzymes, which are released primarily by neutrophils attracted into the area by diverse stimuli.
Frequency
United States
PUK is uncommon. RA has been reported as the most common collagen vascular disorder that causes PUK, accounting for 34% of noninfectious PUK (Eiferman, 1979; Brown, 1968; Tauber, 1990). PUK may be the initial manifestation of WG and PAN. PUK is rare in patients with RP; only 2 of 112 patients with RP were reported to develop PUK in a clinical review study (Hoang-Xaun, 1990). PUK also has been reported to be associated with SLE, although this is uncommon (Messmer, 1999; Watson, 1976). Mooren ulcer is a rare local autoimmune disease associated with PUK, with only 287 cases reported in the world literature, although some of these cases may have been the presenting manifestation of an occult systemic disease rather than true Mooren ulcer (Sangwan, 1997).
Mortality/Morbidity
PUK produces great morbidity from the pain and resultant visual disability. It can be a harbinger of death if the underlying disease is not diagnosed and successfully treated.
Race
No good data are available on racial predilection for PUK.
Sex
Since PUK is more common in people with collagen vascular disorders (especially RA), it is more common in females than in males. However, PUK caused by Mooren ulcer is more common in males than in females (Sangwan, 1997; Tabbara, 1986).
Age
Age varies and is dependent on the associated systemic or local disorder.
History
PUK is frequently a manifestation of an occult systemic disease. Thus, a thorough systemic history is very important and should include chief complaint, characteristics of present illness, past medical history, family history, and a meticulous review of systems (Foster, 1993).
- Ocular symptoms vary, but nonspecific foreign body sensation with or without eye pain, tearing, photophobia, and reduced visual acuity are the most common symptoms for patients with PUK.
- Loss of vision can occur quickly when PUK progresses.
- PUK associated with RA, WG, PAN, and RP often is linked with scleritis, and eye pain may be pronounced in these individuals (Foster, 1993). PUK in patients with Mooren ulcer also may produce pain, although there is no scleral involvement.
- Past medical history and review of systems helps to determine the possible underlying systemic diseases. RA, SLE, PAN, WG, or RP may present with the following symptoms, which should be emphasized in the review of systems (Tabbara, 1986):
- General - Constitutional symptoms, such as chills, fever, poor appetite, recent weight loss, and fatigue
- Skin - Rashes, nodules, vesicles, ulcer, nail changes, and periungual infarcts
- Respiratory - Coughing, wheezing, pneumonia, and shortness of breath
- Cardiac - Chest pain or discomfort and dyspnea
- Gastrointestinal - Abdominal pain, nausea, vomiting, difficulty swallowing, and diarrhea
- Musculoskeletal - Muscle or joint pain, arthritis, back pain, and limitation of motion
- Neurologic - Headaches, seizures, psychiatric, paralysis, and numbness/tingling
- Other systemic symptoms - Deafness, swollen ear lobes, ear infections, vertigo, and noises in ears (suggestive of RP)
Physical
- Examination should be complete and include an overview of the head (including the nose, mouth, and external ear), trunk, joints, and extremities (Foster, 1993). Skin lesions also should be noted.
- A complete ophthalmic examination should be performed with special emphasis on the conjunctiva, sclera, and cornea. Anterior chamber, vitreous, and fundus examinations also are important.
- Patients with PUK typically present with decreased visual acuity (secondary to induced irregular astigmatism), tearing, and eye irritation with or without pain of variable duration (Gregory, 1996).
- Slit lamp examination reveals a crescent-shaped destructive lesion of the juxtalimbal corneal stroma associated with an epithelial defect, stromal yellow-white infiltrates composed of inflammatory cells, and varying degrees of corneal stromal thinning (minimal to full thickness) adjacent to the limbus (Eiferman, 1979).
- In severe cases, the peripheral cornea is progressively destroyed circumferentially and centrally.
- PUK accompanied by necrotizing scleritis almost always indicates the presence of a potentially lethal systemic disease (Foster, 1993).
- The anterior chamber should be evaluated for depth and inflammation.
- A posterior segment examination typically is indicated to help determine the underlying etiology.
Causes
The etiologies for developing PUK are multiple and extensive. Connective tissue and vasculitic diseases are the major risk factors. Other disorders that can cause PUK include systemic and local infectious conditions, as well as local degenerative disorders. The differential diagnosis of PUK includes the following (Foster, 1993; Feldman, 2000):
- Noninfectious conditions
- Systemic - RA, SLE, RP, sarcoidosis, progressive systemic sclerosis, rosacea, WG, PAN, giant cell arteritis, inflammatory bowel disease, and metabolic and neoplastic conditions (Feldman, 2000)
- Local - Mooren ulcer, marginal keratitis, blepharitis (eg, staphylococcal infection, rosacea), contact lens use, chemical injury to the eyes, trauma, surgery, neurotrophic and neuroparalytic causes, nutritional deficiencies, keratoconjunctivitis sicca, Terrien marginal degeneration, pellucid marginal degeneration, and furrow degeneration (Gregory, 1996)
- Infectious conditions
- Systemic - Shigella species, tuberculosis, syphilis, hepatitis, HIV, gonococcus, Salmonella species, and bacillary dysentery
- Local - Herpes simplex keratitis, varicella-zoster keratitis, bacterial keratitis, fungal keratitis, and Acanthamoeba species
Blepharitis, Adult
Giant Cell Arteritis
Herpes Simplex
Herpes Zoster
HIV
Inflammatory Bowel Disease
Keratitis, Bacterial
Keratitis, Fungal
Keratoconjunctivitis, Sicca
Ocular Manifestations of Syphilis
Pellucid Marginal Degeneration
Sarcoidosis
Tuberculosis
Other Problems to be Considered
See Causes.
Lab Studies
- Laboratory tests should focus on the suspected systemic disease (Foster, 1993).
- Complete blood count (CBC)
- Urinalysis (UA), blood urea nitrogen (BUN), and creatinine
- Erythrocyte sedimentation rate (ESR)
- Rheumatoid factor (RF); positive in 80% of patients with associated RA (Foster, 1993; Carson, 1989)
- Angiotensin-converting enzyme (ACE); elevated in sarcoidosis
- Antinuclear antibodies (ANA); positive in patients with SLE and RA
- Antibody to double-stranded DNA (anti-dsDNA); associated with SLE
- Antibodies to small nuclear ribonucleoprotein-Sm (anti-Sm); associated with SLE
- Antibodies to small nuclear ribonucleoproteins-RNP (anti-RNP); associated with SLE
- Antineutrophil cytoplasmic antibodies (ANCA); C-ANCA sensitivity of 96% for active generalized WG, 67% for active regional disease, and 32% for WG in full remission after initial regional symptoms (Foster, 1993; Eiferman, 1979; Ludemann, 1987; Savage, 1987; Nolle, 1989)
- Circulating immune complex (CIC) - Raji cell assay; C-1q binding assay
- Anti-type II antibodies; associated with RP
- Complement - C3 and C4, CH50; consumed in patients with SLE
- Hepatitis B surface antigen (HBsAg); present in 40% of patients with PAN
- Fluorescent treponemal antibody-absorption (FTA-ABS) for syphilis
Imaging Studies
- Chest x-ray and sinus CT scan to rule out WG, sarcoidosis, and tuberculosis
- Radiographic studies of affected joints
Procedures
- Scraping and culture of the ulcer are recommended.
- Conjunctival resection/biopsy may be helpful in removing the limbal source of collagenases and other factors causing progressive ulceration.
Histologic Findings
In Mooren ulcer, corneal thickening occurs at the margin of the ulcer where inflammatory cells have invaded the anterior stromal layers. However, the inflammation is nonspecific, and no etiologic agent can be identified. Necrosis of the involved epithelium and stroma is seen. PUK associated with connective tissue disease and PUK associated with mild infections sometimes may appear similarly. Signs of vasculitis in the adjacent conjunctiva may be seen (Squirrell, 1999).
Medical Care
Local treatment is aimed at preventing or reducing corneal damage, while systemic medication will quiet down the underlying disease.
One goal is to get the epithelial defect to heal, which will halt progressive corneal ulceration.
- Surgical care may be combined with adjunctive local therapy with topical 1% medroxyprogesterone (which inhibits collagenase synthesis) or topical 20% N-acetylcysteine (a competitive inhibitor of collagenase). Lubricating drops, gels, and ointments and antibiotic drops or ointments can be helpful in aiding reepithelialization.
- Topical steroid use is not recommended in the treatment of patients with PUK associated with systemic disease because it may aggravate corneal melt due to collagen synthesis inhibition.
- Systemic collagenase inhibitors (tetracycline 250-mg tab qid or doxycycline 100-mg tab bid) may help slow the progression.
- There is limited experience in the use of topical cyclosporine (Leigner, 1990). Topical cyclosporine combined with lamellar keratoplasty (see Surgical Care) was noted to improve the healing rate in Mooren ulcer (Chen, 2000). However, an underlying systemic vasculitis is not addressed with this route of treatment.
- Many studies have documented that patients with PUK who have associated systemic diseases have recurrences following localized temporizing treatment unless they are given adequate systemic immunosuppressive therapy. To address the underlying problem, both systemic steroid and cytotoxic immunosuppressive medications have been used, alone or in combination, and are effective at controlling ocular and systemic inflammation. Immunosuppressive agents have been indicated for management of the following:
- PUK associated with potentially lethal systemic vasculitic syndromes, such as PAN, RA, SLE, RP, WG, PSS, Sjögren syndrome, allergic angiitis of Churg-Strauss, and giant cell arteritis
- PUK associated with necrotizing scleritis with vasculitis based on histopathologic analysis
- Bilateral and/or progressive Mooren ulcer
- PUK unresponsive to aggressive conventional medical and surgical therapy
- Cyclophosphamide is the drug of choice for almost all PUK associated with connective tissue disorders. The intravenous route has been used with success in PUK associated with rheumatoid arthritis (Clewes, 2005). Methotrexate (MTX), azathioprine, cyclosporine A, and chlorambucil have been found to be effective (Tauber, 1990). High-dose oral prednisone may be started, while the chemotherapeutic agents take effect after 4-6 weeks. When local or systemic infectious causes are suspected, therapy must be aimed at eliminating the infectious organism using the appropriate antibiotic medications based on clinical presentation or culture.
- The use of the tumor necrosis factor alpha (TNF-alpha) antagonist infliximab has been reported to be effective in rheumatoid arthritis-associated PUK cases refractory to the above conventional immunomodulatory therapy (Thomas, 2005).
Surgical Care
- Tissue adhesives, such as cyanoacrylate glue, are recommended for use in impending perforation and perforation size smaller than 1-2 mm (Eiferman, 1979). Adhesive application follows keratectomy and conjunctival resection to remove sources of collagenase, cytokines, and inflammatory cells from the ulcerated cornea, temporarily preventing further stromal loss.
- Application of a bandage contact lens prevents discomfort and dislodging of the adhesive (Eiferman, 1979).
- Amniotic membrane transplantation has been used in the management of Mooren ulcer (Lambiase, 2005). Amniotic membranes have properties that promote rapid healing and reduce ocular surface inflammation (Prabhasawat, 2001). However, they may have a limited role in treating eyes with severe ischemia (eg, rheumatoid arthritis) (Tseng, 2001).
- Tectonic procedures, including lamellar keratoplasty, penetrating keratoplasty, and corneoscleral patch grafts, are performed as needed to maintain the integrity of the globe when corneoscleral perforation is imminent or has occurred.
Consultations
Referral to an appropriate specialist may be necessary. In patients with connective tissue diseases, comanagement with a rheumatologist is necessary to address the systemic disease. Pulmonary, nephrology, cardiac, hematology, and infectious disease consults may be necessary depending on the patient's symptoms and laboratory findings. Regular consultation with an oncologist may be necessary for those patients who are receiving chemotherapy.
Activity
Decreased visual acuity and systemic disorders may be limiting factors.
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Systemic corticosteroids
Have both anti-inflammatory (glucocorticoid) and salt-retaining (mineralocorticoid) properties. Glucocorticoids have profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
| Drug Name | Prednisone (Deltasone, Orasone, Meticorten) |
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| Description | A synthetic glucocorticoid steroid with nonspecific anti-inflammatory and immunosuppressive effects. |
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| Adult Dose | 1 mg/kg/d PO initially; not to exceed 60-80 mg/d; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use; long-term systemic steroids can cause glaucoma and cataracts |
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Drug Category: Immunosuppressive agents
Inhibit key factors that mediate immune response.
| Drug Name | Methotrexate (Folex PFS, Rheumatrex) |
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| Description | A folic acid analog. Acts on the enzyme dihydrofolate reductase, which catalyses the reduction of folate to tetrahydrofolate, a compound necessary for DNA synthesis. Actively replicating cells, such as the leukocyte, are affected and their functions suppressed. |
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| Adult Dose | 7.5-12.5 mg/wk PO/IM/SC single dose initially; not to exceed 40 mg/wk; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency |
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| Interactions | Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX; probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, can increase MTX plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines |
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| Pregnancy | D - Unsafe in pregnancy
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| Precautions | Monitor CBCs monthly; monitor liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occur; fatal reactions reported when administered concurrently with NSAIDs |
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| Drug Name | Azathioprine (Imuran) |
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| Description | A purine nucleoside analog that is activated in the liver producing metabolites, which interfere with purine metabolism. T- and B-cell functions are suppressed. |
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| Adult Dose | 1-3 mg/kg/d PO initially; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; low levels of serum TPMT |
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| Interactions | Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of MTX metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine |
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| Pregnancy | D - Unsafe in pregnancy
|
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| Precautions | Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level prior to therapy; monitor liver, renal, and hematologic function; pancreatitis rarely associated |
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| Drug Name | Cyclosporin A (Sandimmune, Neoral, SangCyA) |
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| Description | An 11-amino acid cyclic peptide and a natural product of fungi. Cyclosporine acts on T-cell replication and activity. |
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| Adult Dose | 2.5-5 mg/kg/d PO divided bid initially; not to exceed 10 mg/kg/d; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UV-B radiation in psoriasis since it may increase risk of cancer |
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| Interactions | Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease cyclosporine concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase cyclosporine toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO |
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| Drug Name | Cyclophosphamide (Cytoxan) |
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| Description | Nitrogen mustard derivative, which affects cell replication by alkylating purines in DNA and RNA. |
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| Adult Dose | Initial dose is 2 mg/kg/d PO; not to exceed 3 mg/kg/d; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; breastfeeding; immunosuppression; leukopenia or thrombocytopenia |
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| Interactions | Long-term treatment with high doses of phenobarbital may increase rate of metabolism and leukopenic activity of cyclophosphamide; allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity; may potentiate effects of succinyl chloride |
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| Pregnancy | D - Unsafe in pregnancy
|
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| Precautions | Bone marrow depression may occur requiring regular blood count monitoring; long-term oral therapy may lead to myelodysplasia; hemorrhagic cystitis is uncommon, but advise all patients to increase fluid intake; monitoring with urinalysis may help by early detection of microscopic hematuria; malignancy, ovarian suppression, testicular atrophy, azoospermia, alopecia, nausea, vomiting, and lymphopenia with resultant opportunistic infections may occur |
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| Drug Name | Chlorambucil (Leukeran) |
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| Description | Slow-acting nitrogen mustard derivative, which interferes with DNA replication, transcription, and nucleic acid function by alkylation. |
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| Adult Dose | 0.1 mg/kg/d PO initially; not to exceed 0.2 mg/kg/d; adjust dose based on clinical response and adverse effects |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; previous resistance to medication |
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| Interactions | None reported |
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| Pregnancy | D - Unsafe in pregnancy
|
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| Precautions | Teratogenic and should be avoided in pregnant women; bone marrow suppression is reversible and may be avoided by routine blood count monitoring; opportunistic infections, nausea, sterility, and amenorrhea may occur; some studies have shown a dose-related increased risk of developing myeloproliferative malignancies later in life |
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Drug Category: Disease modifying agents
These agents alter the immune response to diverse stimuli.
| Drug Name | Infliximab (Remicade) |
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| Description | Chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-a and inhibits its binding to TNF-a receptor. Reduces infiltration of inflammatory cells and TNF-a production in inflamed areas. Used with methotrexate in patients who have had inadequate response to methotrexate monotherapy. |
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| Adult Dose | Follow guideline for treatment of rheumatoid arthritis: 3 mg/kg IV single dose, follow by additional 3 mg/kg at 2 and 6 wk after first dose; repeat q8wk thereafter |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; infections; congestive heart failure |
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| Interactions | None reported |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
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| Precautions | TNF alpha modulates cellular immune responses; anti-TNF therapies, such as infliximab, may adversely affect normal immune responses and allow development of superinfections; more cases of lymphoma were observed in TNF alpha-blockers compared to controlled groups; may increase risk of reactivation of tuberculosis in patients with particular granulomatous infections |
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Further Outpatient Care
- Continued, possibly lifelong, follow-up care is necessary even after complete resolution since relapses may occur. Furthermore, many patients may require prolonged systemic steroid, nonsteroidal anti-inflammatory, and/or chemotherapeutic medications for the systemic disease despite a quiet eye.
Complications
- Ocular complications include corneal scarring and neovascularization with irregular astigmatism, corneal thinning and perforation, loss of vision, and even blindness.
Prognosis
- In patients with collagen vascular diseases, PUK with necrotizing scleritis is associated with poor life expectancy because of the presence of subclinical systemic vasculitis.
Patient Education
- Inform patients about both ocular and systemic components of the disease, treatment options, and possible complications. Emphasize lifelong follow-up care.
- For excellent patient education resources, visit eMedicine's Eye and Vision Center. Also, see eMedicine's patient education article Corneal Ulcer.
Medical/Legal Pitfalls
- Error in diagnosis and treatment
- Failure to explain course of the disease and possible complications
- Failure to refer to proper specialists for systemic symptoms and medications
- Failure to explain possible adverse effects of topical and systemic medications
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Lijing Yao, MD, to the development and writing of this article.
| Media file 1:
Peripheral ulcerative keratitis in the right eye of a patient with rheumatoid arthritis. Glue has been placed. |
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| Media file 2:
Same patient as in Image 1, 1 year posttreatment. |
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| Media file 3:
Left eye of same patient as in Image 1. Note the corneal thinning and scarring. |
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Media type: Photo
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Peripheral Ulcerative Keratitis excerpt Article Last Updated: Jun 2, 2006
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