Reacting to Posterior Scleritis: A Rare Presentation of Reactive Arthritis Marshall Ward (first) Michelle Brown (second) JulieAnne Roper (third) Abstract: A patient with a history of reactive arthritis presents with an aching, blurry left eye. Subsequent appointments result in diagnosis and resolution of posterior scleritis with optic nerve edema and serous macular detachment. I. Case History 1) Patient demographics: 47-year-old Caucasian male 2) Chief complaint: Aching left eye with blurry vision for one week 3) Ocular history: Anterior uveitis, anterior scleritis, and episcleritis 4) Medical history: Reactive arthritis (ReA) with joint pain and psoriasis, hyperlipidemia 5) Medications: atorvastatin calcium 10 mg for cholesterol, mupirocin ointment, naproxen 500 mg for joint pain and ocular ache II. Pertinent Findings: Initial Visit Clinical Findings a) Uncorrected (sc) Visual Acuity (VA): 20/20 OD, 20/30 OS, pinhole (PH) 20/25. Pupils, extraocular muscles (EOM), confrontation visual fields, and Ishihara color testing were normal. Blood pressure was 130/87. Adnexa and slit lamp exam were normal. Intraocular pressures (IOPs) were 17/17. Dilated fundus exam (DFE) and optical coherence tomography (OCT): normal optic nerve head (ONH) OD, edematous ONH 360 OS, deep creamy white circular subretinal lesions OS, shallow macular detachment OS. b) Laboratory Results: CBC, HbA1C, Folate, Vitamin B12, Rheumatoid Factor, FANA, Lyme Titer, ACE, ANCA, HLA-B27, CRP, ESR, RPR, FTA, CCP, methylmalonic acid, Toxoplasmosis IGG/IGM, Quantiferon TB, and Bartonella were all negative for abnormalities. c) Radiology studies: CT studies of the head and orbit with and without contrast were normal. 1) One-Day Follow Up Clinical Findings a) Sc VA: 20/40 OS PH 20/25. Pupils: 1+ RAPD OS. EOMs: slight pain OS on eye movement. HVF 30-2 revealed slight depression superiorly OS, otherwise normal. DFE: unchanged from previous exam. Ophthalmology was consulted and agreed to re-examine patient in 1 day and agreed to continue current naproxen therapy. 2) Two-Day Follow Up Clinical Findings a) Sc VA: 20/80+2 OS, PH no improvement. DFE and OCT: stable ONH edema OS, increase in macular thickness/macular detachment, new flame shaped hemorrhages between disc and macula. B-scan showed scleral darkening, but no scleral thickening or T sign. b) Meds: Start Prednisone 80 mg QD PO x 1 week. Start acyclovir 800 mg 5x/day x 1 week to cover for serpiginous associated to herpes virus. 3) One-Week Follow Up Clinical Findings a) Sc VA: 20/25-1 OS. DFE and OCT: improvement in ONH edema OS with no hemorrhages, resolution of deep white lesions, and decrease in macular thickness/macular detachment. b) Meds: Taper Prednisone by 20 mg q2 days. Continue acyclovir 800 mg 5x/day x 1 week. 4) Two-Week Follow Up Clinical Findings a) Sc VA: 20/40+2 OS. DFE and OCT: improvement in ONH edema. b) Meds: Ibuprofen 600mg PO TID 5) Six-Week Follow Up Clinical Findings a) Sc VA: 20/40 PH NI OS. DFE and OCT: resolution of ONH edema and macular detachment OS. III. Differential diagnosis 1) Primary: Posterior Scleritis (PS) – Inflammation of the posterior sclera leading to optic disc edema, vision loss, and pain. Often associated with seronegative spondyloarthropathies such as ReA. 2) Others: Neuroretinitis, serpiginous choroidopathy, optic neuritis, toxoplasmosis, acute retinal necrosis, progressive outer retinal necrosis IV. Diagnosis and discussion 1) Elaborate on the condition: PS is inflammation of the posterior sclera. The mean age of onset is 49.3 years (1). Presentation is unilateral in 65% of cases. Symptoms include vision loss and pain. Clinical signs may include choroidal folds, serous retinal detachment, optic disc edema, subretinal granuloma, a “T” sign on ultrasonography, and proptosis (2). PS is often associated with auto- immune disorders, but can also occur secondary to infectious conditions such as herpes zoster virus, Lyme, tuberculosis, and syphilis (3). 2) Unique features: While anterior segment inflammation is commonly seen in patients with ReA, PS is a rare manifestation of the disease (4,5). Furthermore, only 25% of patients with ReA are HLA- B27 negative (6). V. Treatment, management 1) Systemic steroids and NSAIDs are mainstays of treatment for auto-immune related PS. Prednisone is dosed 60-100mg PO q.d. for 1 week, followed by taper to 20mg q.d. over the next 2-6 weeks (3). NSAIDs such as ibuprofen 400-600mg p.o. q.i.d, naproxen 250-500mg p.o. b.i.d., or indomethacin 25mg p.o. t.i.d. can be used (7). Multiple NSAIDs can be tried if the first is unsuccessful. If NSAIDs and steroids prove unsuccessful, immunosuppressive therapy is indicated (7). 2) Research/Literature review/ Bibliography: References are attached at the end of the outline and the findings in research and review of the literature are mentioned in the discussion and treatment sections of this outline. VI. Conclusion 1) Though rare, PS should be considered in a patient with an autoimmune condition such as ReA that presents with ONH edema, gradual vision loss, and boring ocular pain. Likewise, autoimmune conditions should be considered as a systemic diagnosis in patients that present with PS. 2) With timely intervention, we were able to treat PS and avoid further vision loss. References 1. Mccluskey PJ, Watson PG, Lightman S, Haybittle J, Restori M, Branley M. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology. 1999;106(12):2380-6. 2. Bowling B. Kanski's Clinical Ophthalmology, A Systematic Approach. 2016. 3. Gerstenblith AT, Rabinowitz MP. The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease. Lippincott Williams & Wilkins; 2012. 4. Kiss S, Letko E, Qamruddin S, Baltatzis S, Foster CS. Long-term progression, prognosis, and treatment of patients with recurrent ocular manifestations of Reiter's syndrome. Ophthalmology. 2003;110(9):1764-9. 5. Brygida Kwiatkowska and Maria Maślińska (2011). Ocular Symptoms (Conjunctivitis, Uveitis) in Reactive Arthritis, Conjunctivitis - A Complex and Multifaceted Disorder, Prof. Zdenek Pelikan (Ed.), InTech 6. Mansour AM, Jaroudi MO, Medawar WA, Tabbarah ZA. Bilateral multifocal posterior pole lesions in Reiter syndrome. BMJ Case Rep. 2013;2013 7. Lavric A, Gonzalez-lopez JJ, Majumder PD, et al. Posterior Scleritis: Analysis of Epidemiology, Clinical Factors, and Risk of Recurrence in a Cohort of 114 Patients. Ocul Immunol Inflamm. 2016;24(1):6-15.