History of Present Illness
A 54-year-old Caucasian female with long-standing rheumatoid arthritis complains of severe right eye (OD) pain and redness for 1 week. The symptoms respond minimally to ibuprofen. She has had similar pain in both of her eyes in the past couple of years and was treated with topical corticosteroid drops and oral nonsteroidal antiinflammatory drugs (NSAIDs) by her ophthalmologist.
Her current medications include methotrexate 15 mg by mouth (PO) weekly, prednisone 7.5 mg PO daily, and ibuprofen 400 mg every 4 to 6 hours as needed ( Fig. 17.1 ).
| OD | OS | |
|---|---|---|
| Visual acuity | 20/100 | 20/20 |
| Intraocular pressure (IOP) | 14 | 14 |
| Sclera/conjunctiva | 3+ superior conjunctival injection with necrotic and avascular sclera | Diffuse scleral thinning nasally |
| Cornea | Moderate temporal haze without thinning | Clear |
| Anterior chamber (AC) | Trace cells | Deep and quiet |
| Iris | Unremarkable | Unremarkable |
| Lens | 1+ nuclear sclerosis (NS) and trace posterior subcapsular cataract (PSC) | 1+ NS |
| Anterior vitreous | Clear | Clear |
Questions to Ask
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How are your joint symptoms?
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Have you had any upper or lower respiratory tract problems, including nosebleed, sinus pain, or bloody sputum?
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Any pain in your earlobes?
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Any known kidney problem?
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Have you had ocular rosacea or severe blepharitis in the past?
She states that her rheumatoid arthritis has gradually worsened despite systemic therapy, the dose of which has remained the same for the past year. She denies upper respiratory tract or pulmonary symptoms. There is no known nephropathy.
Assessment
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Necrotizing scleritis OD with evidence of past scleritis left eye (OS)
Differential Diagnosis
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Rheumatoid arthritis
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Granulomatosis with polyangiitis (GPA, or formerly Wegener granulomatosis)
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Polyarteritis nodosa
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Systemic lupus erythematosus
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Relapsing polychondritis
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Ocular rosacea
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Atopy
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Herpesviruses
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Tuberculosis
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Syphilis
Working Diagnosis
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Necrotizing scleritis secondary to rheumatoid arthritis
Testing
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In this case, the patient had a known diagnosis of rheumatoid arthritis, so the scleritis is presumed to be related to this. However, when encountering a patient with necrotizing scleritis without known systemic disease, the following laboratory and radiographic testing should be conducted:
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Rheumatoid factor, anti-citrulline peptide antibodies
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Antinuclear antibodies (ANA)
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Antineutrophil cytoplasmic antibodies (ANCA)
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Circulating immune complexes
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Complement levels
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QuantiFERON, purified protein derivative (PPD)
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Fluorescent treponemal antibody absorption (FTA-ABS), rapid plasma reagin (RPR)
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Herpes simplex virus (HSV) and varicella zoster virus (VZV) immunoglobulin G (IgG) and immunoglobulin M (IgM)
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Sinus and chest radiographs
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Blood urea nitrogen (BUN)/creatinine, urinalysis
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For cases resistant to antiinflammatory therapy, scleral biopsy should be considered to look for immunofluorescent staining for anti-HSV or VZV antibodies
Management
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Prednisone was increased immediately to 60 mg every day (QD)
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Methotrexate was discontinued, and intravenous cyclophosphamide commenced immediately
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Careful monitoring for scleral or corneal perforation
Follow-up
The patient’s scleritis became quiescent after alkylating therapy and slow prednisone taper. Her cyclophosphamide was discontinued after 9 months, and she eventually resumed methotrexate, but at a higher dose given subcutaneously. She subsequently underwent scleral patch surgery for reinforcement ( Fig. 17.2 ).
