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S. S. Bhullar, P. Lin, H. H. Tessler, D. A. Goldstein; Immunological Markers as Potential Predictors of Systemic Autoimmune Disease in Idiopathic Scleritis Patients. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5836.
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To determine the usefulness of immunological marker testing in patients with idiopathic scleritis (IS).
Medical records of all patients with idiopathic scleritis seen at the University of Illinois Uveitis clinic between August of 1995 and August 2006 were reviewed. Ophthalmic and medical histories, ophthalmic exam and results of lab tests including ANCA, ANA, FTA-ABS, and RF were collected.
128 consecutive patients with a diagnosis of scleritis were identified. 88 (68%) were classified as having idiopathic scleritis at initial presentation. Seventy one patients with idiopathic scleritis had rheumatoid factor (RF) titers recorded, 20 (23%) were positive. 10 (50%) of the patients who tested positive for RF were ultimately diagnosed with rheumatoid arthritis (RA) with a mean follow-up of 10.0 months. 7 (13.7%) of patients with RF negative titers developed rheumatologic diagnoses including RA (2%), Wegener's granulomatosis (WG) (6%), relapsing polychondritis (2%), and spondylarthropathy (4%). The corrected odds ratio for developing RA in patients with idiopathic scleritis and a positive RF was 50 compared to patients who present with scleritis and a negative RF (95% CI 5.738 - 435.68, p<0.0001). Sixty nine patients had ANCA titers available, 7 (10%) were positive. 3 (43%) of these patients were ultimately diagnosed with WG. 2 (3%) of patients initially tested negative for ANCA developed WG during a mean follow up of 9.2 months. The odds ratio for a diagnosis of WG in patients with idiopathic scleritis and a positive ANCA screen at the initial visit is 13.3 compared to patients who present with idiopathic scleritis and a negative ANCA screen (95% CI 2.214 - 79.149, p=0.016).
An RF titer is a useful screening tool in patients with idiopathic scleritis. Scleritis may be the initial presentation of RA, particularly in patients with elevated RF levels. There may also be a role for repeated RF titers in patients who initially test negative for RF. This study also supports the role of ANCA screening for WG in patients with idiopathic scleritis. Although the diagnosis of RA and WG are typically based on the presence of multi-organ or polyarticular involvement, abnormal RF and ANCA screens in the setting of idiopathic scleritis identify an at-risk patient population.
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