Study Highlights Influence of Systemic and Infectious Diseases in Scleritis Cases

This study underlines the diagnostic value of specific ANCA testing for scleritis patients, while repeating laboratory investigations without new clinical manifestations appears to be of limited use. Photo: Katherine Sanford, OD. Click image to enlarge. Scleritis is a rare but troubling finding, and often suggests a systemic origin that must be determined by the clinician. In a recent study, researchers assessed the contribution of systemic workup and long-term follow-up in such patients. They found that anti-neutrophil cytoplasmic antibody (ANCA) testing may be of value. The findings were reported in Journal of Ophthalmic Inflammation and Infection.This retrospective study reviewed 98 patients with scleritis who had no known pre-existing disease. After a median follow-up of 42 months, an associated disease was identified in 37.8% of patients, comprising 23.5% with systemic diseases, 12.2% with infectious diseases and two cases of drug-induced scleritis (2%).The positive predictive value of non-specific ANCA testing was 29%, while its negative predictive value was 98%. When specific ANCA testing was performed, the positive predictive value increased to 86%. Repeating laboratory investigations during follow-up didn’t yield additional diagnoses. This study highlights the importance of a systemic evaluation in patients with scleritis of initially unknown origin. “Our findings suggest that such an approach can reveal an associated disease in approximately 40% of patients—most often systemic—either at baseline or during fol­low-up,” the authors wrote in their paper. “While these results support the usefulness of a structured systemic assessment, they remain observa­tional and should not be interpreted as proving causality.”In 20% of patients with scleritis referred to the internal medicine department, positive findings played a key role in identifying an associated systemic disease, mainly through ear, nose and throat (ENT) and dermatological assessments. These results support the value of structured systemic assessment, the authors noted in their paper. In 70% of these patients, systemic manifestations preceded the first episode of scleritis, while in the remaining 30% they developed during follow-up. Scleritis was the initial and sole manifestation in three patients, with the diagnosis established through additional investigations during follow-up. This sequential diagnostic process underscores that many systemic diseases associated with scleritis evolve over time and that a single baseline evaluation may be insufficient, thereby highlighting the value of lon­gitudinal follow-up.Half of the associated diseases were identified during follow-up. These diagnoses arose through four mecha­nisms: emergence of new systemic manifestations, new laboratory or imaging findings, new ophthalmologic examinations or response to empirical treatment. This sequential diagnostic process underscores that many sys­temic diseases associated with scleritis evolve over time and that a single baseline evaluation may be insufficient. An associated systemic disease was identified at the initial examination in most patients, based on pre-existing systemic manifestations. In others, it was diagnosed later, following the appearance of new sys­temic manifestations, up to 12 years after the first epi­sode. In nearly 15% of patients, additional imaging or ophthalmologic examinations contributed to the identifi­cation of the systemic disease. Conversely, in infectious diseases, no pre-existing sys­temic manifestations were found. In approximately 40% of cases, diagnosis relied mainly on ocular course and therapeutic response, highlighting the difficulty of dis­tinguishing infectious from immune-mediated scleritis based solely on baseline evaluation.Additionally, this study suggests the importance of focusing on ENT manifestations and a dermatological examination. It also highlights the value of testing for ANCA and determining its specificity in the initial assessment of scleritis. Repeating laboratory tests in the absence of new clinical manifestations in idiopathic scleritis appears lim­ited; however, prospective studies assessing standardized strategies for the identification of associated systemic diseases in scleritis are warranted, the authors concluded in their paper.Click here for the journal source. Soubrier M, Vasseneix C, Jacquoit R, et al. Scleritis and associated systemic diseases: contribution of systemic examination, follow-up and additional investigations. J Ophthalmol Inflamm Infect. [Epub ahead of print]. This article was developed by the editorial staff in conjunction with experts in the field. In the process, AI may have been among the editorial tools used to meet the goals of human editors, who approved all content.