Karoline Baquet-Walscheid

Background: Juvenile idiopathic arthritis-associated uveitis (JIAU) frequently follows a chronic and complicated course. Janus kinase inhibitors (JAKi) represent a promising new therapeutic option. Methods: Retrospective monocentric analysis of 20 children with chronic anterior JIAU treated with tofacitinib ( n = 18), baricitinib ( n = 1) or upadacitinib ( n = 1). Outcome measures: uveitis or arthritis inactivity at any time during follow-up, recurrences after achieving inactivity, sparing of medication, occurrence of new ocular complications, resolution of preexisting macular edema. Results: All patients had received methotrexate prior to baseline, and at least two biologics had been ineffective in 18/20 patients. Whilst receiving JAKi medication, uveitis was inactive in eight patients (anterior chamber cell count < 0.5+) at any time during follow-up (mean 9.2 ± 3.3 months), but uveitis relapsed in 6 of them subsequently. In 7 patients, treatment was terminated after ≤ 1 year due to inadequate response of ocular inflammation. During follow-up, new uveitis-related complications occurred in 7 patients (9 eyes), the most frequent were macular edema (8 eyes of 7 patients), and ocular hypertension (5 eyes of 3 patients). Reduction of concomitant systemic medication was possible in 3/14 patients. Any reduction of topical steroids during follow-up was possible in 24/30 eyes, and 23/30 eyes received a lower dose at the last follow-up than they did at baseline. Conclusions: Our data show limited effectiveness of JAKi treatment in patients with chronic DMARD-refractory JIAU. The number of patients in whom uveitis quiescence could be observed was low, as was the possibility for reduction of concomitant medication, and the relapse rate was high. Clinical trial number: Not applicable.

In the above-mentioned article, a citation was mistakenly omitted on page 638. This should have referenced “Baquet-Walscheid K, Rath T, Heiligenhaus A. Augenbeteiligung bei ausgewählten rheumatischen Erkrankungen – Klinik im Erwachsenenalter. Klin Monbl Augenheilkd 2024; 241: 626-632. doi: 10.1055/a-2239-0412”. The issue has now been corrected. Article published online: 22 May 2024 © 2024. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Peripheral ulcerative keratitis (PUK) is an inflammatory disease of the peripheral cornea, which may frequently be associated with several rare, but potentially life-threatening systemic diseases. The inflammatory pathogenesis of PUK results from humoral and cell-mediated inflammation. The diagnosis is usually based on the typical clinical findings and always requires detailed diagnostic testing to identify a potential systemic underlying disease. Treatment includes topical and systemic immunosuppressive and immunomodulatory therapeutic strategies and, in the event of impending or existing perforation, also various surgical interventions. PUK is a potentially blinding disease that initially affects the periphery, but, if left untreated, can lead to destruction of the entire cornea. Interdisciplinary diagnostic testing and therapy are crucial to preserve vision in the affected patients and reduce morbidity and mortality. The following article provides an overview of the pathophysiology, clinical findings, possible underlying systemic diseases, relevant differential diagnoses and therapeutic strategies.

Ocular manifestations of rheumatic diseases are common and contribute significantly to the morbidity and reduced quality of life of affected patients. Knowledge of typical clinical manifestations is important for the rheumatologist in order to support the reference of patients with corresponding symptoms for ophthalmological consultation at an early stage of disease, or to initiate regular screening examinations (e.g. in patients with Behçetʼs syndrome). Conversely, a (possibly urgent) rheumatological assessment is crucial for certain ophthalmological diseases, in order not to overlook a (possibly fatal) systemic associated disease. Patients with rheumatic or inflammatory ocular diseases should always be informed by the treating physician about possible symptoms of other organ manifestations, in order to avoid a delayed diagnosis. “Classic” associations for uveitis are (HLA-B27-associated) spondyloarthritis and acute anterior uveitis, as well as retinal vasculitis with or without panuveitis and Behçetʼs syndrome. In patients with rheumatoid arthritis or ANCA-associated vasculitis, however, scleritis (with or without peripheral ulcerative keratitis) typically occurs, but a variety of other findings are also possible. Close interdisciplinary collaboration, particularly regarding therapeutic decisions, is crucial to ensuring a good prognosis for the patient.

Intermediäre und posteriore Uveitis können multiple infektiöse und nicht infektiöse Ursachen haben, und insbesondere posteriore Uveitiden sind klinisch sehr vielgestaltig. Einige Entitäten bedürfen einer umgehenden Therapieeinleitung, um die Visusprognose zu sichern. Der Beitrag zeigt typische Charakteristika intermediärer und posteriorer Uveitiden und erläutert Besonderheiten bei deren Behandlung. Intermediate and posterior uveitis can have multiple infectious and noninfectious causes, and posterior uveitis in particular is clinically multifaceted. Some entities require prompt initiation of therapy to ensure visual prognosis. This article presents typical characteristics of intermediate and posterior uveitides and explains special features of their treatment. Article published online: 17 November 2023 © 2023. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Intermediäre und posteriore Uveitis können multiple infektiöse und nicht infektiöse Ursachen haben, und insbesondere posteriore Uveitiden sind klinisch sehr vielgestaltig. Einige Entitäten bedürfen einer umgehenden Therapieeinleitung, um die Visusprognose zu sichern. Der Beitrag zeigt typische Charakteristika intermediärer und posteriorer Uveitiden und erläutert Besonderheiten bei deren Behandlung. Intermediate and posterior uveitis can have multiple infectious and noninfectious causes, and posterior uveitis in particular is clinically multifaceted. Some entities require prompt initiation of therapy to ensure visual prognosis. This article presents typical characteristics of intermediate and posterior uveitides and explains special features of their treatment. Article published online: 17 November 2023 © 2023. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Patients with juvenile idiopathic arthritis (JIA) associated uveitis (JIAU) are at risk for secondary glaucoma, frequently requiring surgical management. We compared the success rates for trabecule...

Objective: The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to update the current guidelines, and the objective here was to produce this document to guide clinicians managing children with juvenile idiopathic arthritis–associated uveitis (JIAU) and idiopathic chronic anterior uveitis (CAU).Methods: The group analyzed the literature published between December 2014 and June 2020 after a systematic literature review conducted by 2 clinicians. Pediatric rheumatologists were paired with ophthalmologists to review the eligible 37 publications. The search criteria were selected to reflect those used for the 2018 Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, in order to provide an update, rather than a replacement for that publication. The summary of the current evidence for each SHARE recommendation was presented to the expert committee. These recommendations were then discussed and revised during a video consensus meeting on January 22, 2021, with 14 voting participants, using a nominal group technique to reach consensus.Results: JIAU treatment was extended to include CAU. Fourteen recommendations regarding treatment of JIAU und CAU with >90% agreement were accepted.Conclusion: An update to the previous 2018 SHARE recommendations for the treatment of children with JIAU with the addition of CAU was created using an evidence‐based consensus process. This guideline should help support clinicians to care for children and young people with CAU.

Noninfectious scleritis typically takes a chronic course, and systemic corticosteroids or disease-modifying anti-rheumatic drug (DMARD) treatment may be inevitable for a prolonged period. Janus kinase (JAK) inhibitors are a relatively new therapeutic option for inflammatory diseases, and three cases of successful treatment of scleritis with tofacitinib, a substance targeting JAK-1 and -3, have been published up to now. We here describe the case of a 51-years-old female patient with bilateral anterior and posterior scleritis who, after treatment failure of multiple DMARDs, finally achieved clinical quiescence of disease under treatment with upadacitinib, a selective JAK-1 inhibitor.

Several case reports have been published on the effect of janus kinase inhibitors (JAK-I) on juvenile idiopathic arthritis-associated uveitis (JIAU). Both tofacitinib and baricitinib have been desc...