1 Jakob, et al: Epidemiology of uveitis Uveitis Subtypes in a German Interdisciplinary Uveitis Center — Analysis of 1916 Patients EVA JAKOB, MIRJAM S. REULAND, FRIEDERIKE MACKENSEN, NADINE HARSCH, MONIKA FLECKENSTEIN, HANNS-MARTIN LORENZ, REGINA MAX, and MATTHIAS D. BECKER ABSTRACT. Objective. Studies on the epidemiology of uveitis are rare and cohorts are small. We analyzed the frequencies of classified forms of uveitis in all patients at our center. Methods. We studied 1916 consecutive patients with inflammatory eye disease. Data were analyzed regarding associated systemic disease, infection, ocular syndromes, anatomic localization, age, and sex. Results. In 59.1% of patients, a classified form of uveitis was observed: associated systemic diseases in 43.7%, the most frequent ones sarcoidosis (17.4%) and ankylosing spondylitis (16.8%); ocular syndromes in 34.3%, the most frequent HLA-B27-positive anterior uveitis (AU; 35.1%) and Fuchs uveitis syndrome (FUS; 34.3%); and infections in 22.4%, the most frequent herpetic infections (46.1%) and toxoplasmosis (31.5%). We found AU in 45.4% of patients (15.4% HLA-B27-positive AU and 11.3% FUS), intermediate uveitis in 22.9% (unclassified 53.7% and multiple sclerosis 10.3%), and posterior uveitis in 13.5% (24.7% toxoplasmosis). Panuveitis was diagnosed in 6.2% of cases (Behçet’s disease 12.6%; sarcoidosis 10.9%). The remaining 12.0% of cases showed extrau- veal manifestations (scleritis, episcleritis, keratitis, optic neuritis, myositis, and orbital inflamma- tion). Conclusion. We describe the largest cohort to date of consecutive patients from a specialized uveitis center. The high frequency of classified disease, nearly 60% in our clinic, shows the usefulness of an interdisciplinary approach, oriented on anatomic presentation. (J Rheumatol First Release Dec 15 2008; doi:10.3899/jrheum.080102) Key Indexing Terms: UVEITIS EPIDEMIOLOGY DIAGNOSIS OCULAR INFLAMMATION From the Interdisciplinary Uveitis Center, University Eye Hospital; and the Interdisciplinary Uveitis Center, Department for Internal Medicine V, University of Heidelberg, Heidelberg, Germany. Dr. Jakob and Dr. Reuland contributed equally to this report. E. Jakob, MD; M.S. Reuland, MD, Resident; F. Mackensen, MD, Ophthalmologist; N. Harsch, MD, Resident; M. Fleckenstein, MD, Resident, Interdisciplinary Uveitis Center, University Eye Hospital; H. Lorenz, MD, Professor of Rheumatology; R. Max, MD, Rheumatologist, Interdisciplinary Uveitis Center, Department for Internal Medicine V; M.D. Becker, Professor of Ophthalmology, Interdisciplinary Uveitis Center, University Eye Hospital. Address reprint requests to Dr. F. Mackensen, Interdisciplinary Uveitis Center, University Eye Hospital, INF 350, Heidelberg 69120, Germany. E-mail: Friederike.Mackensen@uveitiszentrum.de Accepted for publication August 29, 2008. Uveitis, the term used for intraocular inflammation of the uvea, is responsible for 5%–20% of cases of legal blindness and 10% of severe visual disorders in the developed nations 1-7 . Frequently, the diagnosis of uveitis conceals ocu- lar involvement related to a systemic disease, an infection, or an ocular syndrome. The specific diagnosis of the under- lying disease is difficult to determine and requires interdis- ciplinary cooperation. Often rheumatologists are asked by an ophthalmologist to “investigate” a patient with uveitis for a rheumatic disease, which is a challenge for the rheumatol- ogist, as uveitis is one diagnosis with heterogeneous subsets that correspond to many diseases 8 . Extensive and costly lab- oratory and imaging diagnostics are often ordered without giving any result. In order to diagnose associated conditions and recognize treatment necessities without squandering healthcare resources, knowledge of the epidemiology of uveitis is essential. However, studies on the epidemiology of uveitis are rare and usually include small cohorts; and the results are controversial, due to differences in numbers of study participants, geography, quality of classification sys- tem, and patient selection. Progress has been made in recognizing, diagnosing, and classifying uveitis and associated systemic disease. Thus, older epidemiologic studies have their limitations. A precise analysis makes possible an effective approach that incorpo- rates likelihoods of associated diagnoses depending on anatomic localization and patient’s sex and age group. We describe the biggest cohort of patients with uveitis from one tertiary referral center published to date, and illustrate the usefulness of this diagnostic approach. MATERIALS AND METHODS We performed a retrospective analysis of the medical records of all patients who presented to the Interdisciplinary Uveitis Center at the University of Heidelberg between October 2001 and October 2006. Inclusion criteria were a diagnosis of uveitis, or extrauveal disease as defined below.