Clinical Review & Education

JAMA Ophthalmology Clinical Challenge

A Painful Red Eye Xiongfei Liu, BS; Ajay E. Kuriyan, MD; Guillermo Amescua, MD

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B

Figure. A, Slitlamp photograph of the patient’s right eye at presentation. B, Anterior segment ultrasonography of the right eye at presentation.

A man in his 40s with a history of sinusitis presented with a 2-month history of worsening pain and redness of the right eye and nasal congestion. He was previously diagnosed with conjunctivitis and treated empirically with artificial tears, ofloxacin drops (0.3%), and prednisolone acetate drops (1%) 4 times a day, without improvement. His visual acuity was 20/20 OD. Examination findings of the anterior segment of the right eye revealed a raised, nonmobile, engorged perilimbal nodule in the inferotemporal sclera that was tender to palpation and did not blanch with phenylephrine drops (2.5%) (Figure, A). The ultrasonogram demonstrated a noncystic, regular, dome-shaped lesion with medium to high reflectivity localized to the sclera without intraocular involvement (Figure, B). The remaining results of the right-eye examination and the entire left-eye examination were within the reference range. In addition, the patient had a right-sided intranasal mass. Laboratory test results demonstrated elevated inflammatory markers, with an erythrocyte sedimentation rate of 38 mm/h (reference range, 0-20 mm/h) and a C-reactive protein level of 190 mg/L (reference range, 0.08-3.1 mg/L) (to convert to nanomoles per liter, multiply by 9.524). Results of serum antineutrophil cytoplasmic antibody (ANCA), rheumatoid factor, antinuclear antibody, angiotensin-converting enzyme, fluoQuiz at jamaophthalmology.com rescent treponemal antibody absorption, rapid plasma reagin, and tuberculosis skin testing were within their reference ranges. Results of urine microscopy had 4+ red blood cells per high-power field. His blood urea nitrogen and creatinine levels were within their reference ranges.

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WHAT WOULD YOU DO NEXT?

A. Increase topical corticosteroid frequency B. Inject sub–Tenon capsule triamcinolone acetonide C. Give oral prednisone D. Refer to otolaryngologist for nasal mass biopsy

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Clinical Review & Education JAMA Ophthalmology Clinical Challenge

Diagnosis Nodular scleritis secondary to Wegener granulomatosis, or granulomatosis with polyangiitis

What To Do Next D. Refer to otolaryngologist for nasal mass biopsy

Discussion The patient’s presentation was consistent with nodular scleritis, which is associated with an underlying systemic disorder in 50% of patients.1 Therefore, a comprehensive evaluation for systemic diseases is essential in the management of scleritis. Although results of the initial examination showed no abnormalities, his sinusitis with nasal mass and microscopic hematuria were indicative of a systemic disorder. This prompted referral to an otolaryngologist for biopsy of the mass. Topical corticosteroids do not adequately penetrate to the sclera, limiting their effectiveness in scleritis.2 Because the major infectious etiologies (syphilis and tuberculosis) were ruled out with laboratory testing, local or oral corticosteroids were therapeutic options.2 However,systemicnonsteroidalanti-inflammatorymedicationscanbeused as first-line treatment in nonnecrotizing and nonposterior scleritis.2 While awaiting laboratory and biopsy results, the patient was given 800 mg of oral ibuprofen 3 times daily, but his symptoms did not improve. Once the major infectious etiologies (syphilis and tuberculosis) were excluded, oral prednisone, 60 mg/d, was given. There was moderate improvement in the size and injection of the nodule and pain at his 2-week follow-up. At this point, the biopsy results were available and demonstrated a polymorphous lymphohistiocytic and eosinophilic infiltrate. On the basis of his sinusitis, microhematuria, and nasal biopsy findings, we, along with our colleagues in the rheumatology department, diagnosed the patient’s condition as ANCA-negative granulomatosis with polyangitis (GPA). Consequently, he was given 20 mg of oral methotrexate sodium weekly and his oral prednisone dosage was decreased to 20 mg/d. Granulomatosis with polyangitis has an incidence of 5 to 10 per 1 million patients and is characterized by vasculitis, glomerulonephritis, and necrotizing granulomatous inflammation of the ARTICLE INFORMATION

Patient Outcome At his 1-month follow-up, there was a dramatic decrease in the size of the nodule and he reported resolution of pain. His oral prednisone dosage was gradually tapered off and he continued taking methotrexate weekly. At his 12-month follow-up, he had stable, nonelevated, painless, minimal injection of the inferior temporal quadrant of the right eye with adjacent pseudopterygium secondary to local limbal stem cell deficiency.

2. Sims J. Scleritis. Postgrad Med J. 2012;88(1046): 713-718.

Author Affiliations: Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miami, Florida.

3. Scott DG, Watts RA. Systemic vasculitis. Ann Rheum Dis. 2000;59(3):161-163.

Corresponding Author: Ajay E. Kuriyan, MD, Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, 900 NW 17th St, Miami, FL 33136 ([email protected]).

4. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101-1107.

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

5. Harman LE, Margo CE. Wegener’s granulomatosis. Surv Ophthalmol. 1998;42(5): 458-480.

REFERENCES 1. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with rheumatoid arthritis and with other systemic immune-mediated diseases. Ophthalmology. 1994;101(7):1281-1288.

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respiratory tract.3 The American College of Rheumatology 1990 criteria for the classification of GPA requires 2 of the following: (1) nasal or oral inflammation, (2) abnormal findings on chest radiography, (3) microhematuria, or (4) granulomatous inflammation detected by biopsy.4 The diagnosis of GPA is based on a combination of clinical findings, positive results of ANCA serologic testing, and histological findings.5 Cytoplasmic ANCA has a sensitivity and specificity of 91% and 99%, respectively, making it a useful tool in GPA diagnosis.6 However, only 84% of patients with active GPA will have positive ANCA titers.7 As highlighted by this case, in the setting of ANCA-negative serologic results and clinical signs indicative of GPA, biopsy is an especially valuable component of the diagnostic criteria. Given GPA’s multiorgan manifestations, it is important for an ophthalmologist considering the diagnosis to do a more extensive examination so as not to miss findings like this patient’s intranasal mass. Ophthalmic involvement occurs in 50% of patients with GPA and is a significant cause of morbidity.8 Scleritis or episcleritis accounts for approximately 12% of GPA ophthalmic manifestations.9 A thorough examination to rule out any disease processes that would benefit from long-term immunosuppression is imperative in all scleritis cases.9 Patients with GPA who have severe multisystem involvement are often treated with cyclophosphamide, while milder, more localized manifestations are treated with azathioprine sodium or methotrexate. Patients with severe GPA treated with corticosteroids alone have a mean survival of 1 year compared with a 93% remission rate with cyclophosphamide.10 Therefore, identifying GPA as the cause of scleritis in patients can lead to changes in management that greatly affect prognosis.

6. Rao JK, Weinberger M, Oddone EZ, Allen NB, Landsman P, Feussner JR. The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis. Ann Intern Med. 1995;123(12):925-932.

7. Specks U, Wheatley CL, McDonald TJ, Rohrbach MS, DeRemee RA. Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener’s granulomatosis. Mayo Clin Proc. 1989;64(1):28-36. 8. Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener’s granulomatosis. Surv Ophthalmol. 2010;55(5):429-444. 9. Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocular manifestations of Wegener’s granulomatosis. Am J Med. 1977;63(1):131-141. 10. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis. Ann Intern Med. 1983; 98(1):76-85.

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A painful red eye.

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