Objective To develop recommendations for the screening, monitoring, and treatment of uveitis in children with juvenile idiopathic arthritis (JIA). Methods Pediatric rheumatologists, ophthalmologists with expertise in uveitis, patient representatives, and methodologists generated key clinical questions to be addressed by this guideline. This was followed by a systematic literature review and rating of the available evidence according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. A group consensus process was used to compose the final recommendations and grade their strength as conditional or strong. Results Due to a lack of literature with good quality of evidence, recommendations were formulated on the basis of available evidence and a consensus expert opinion. Regular ophthalmic screening of children with JIA is recommended because of the risk of uveitis, and the frequency of screening should be based on individual risk factors. Regular ophthalmic monitoring of children with uveitis is recommended, and intervals should be based on ocular examination findings and treatment regimen. Ophthalmic monitoring recommendations were strong primarily because of concerns of vision-threatening complications of uveitis with infrequent monitoring. Topical glucocorticoids should be used as initial treatment to achieve control of inflammation. Methotrexate and the monoclonal antibody tumor necrosis factor inhibitors adalimumab and infliximab are recommended when systemic treatment is needed for the management of uveitis. The timely addition of nonbiologic and biologic drugs is recommended to maintain uveitis control in children who are at continued risk of vision loss. Conclusion This guideline provides direction for clinicians and patients/parents making decisions on the screening, monitoring, and management of children with JIA and uveitis, using GRADE methodology and informed by a consensus process with input from rheumatology and ophthalmology experts, current literature, and patient/parent preferences and values.

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2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis–Associated Uveitis

To present our preliminary experience with JAK inhibitors in treating patients affected by juvenile idiopathic arthritis (JIA) and associated uveitis. Case series. Four consecutive patients with long-term history of juvenile idiopathic arthritis and severe associated uveitis were included in the study. Indication for treatment with JAK inhibitors was uncontrolled arthritis and/or uveitis despite different treatments with conventional and biologic disease modifying antirheumatic drugs (DMARDs). While on treatment with JAK inhibitors, namely, baricitinib (three cases) and tofacitinib (one case), all our patients showed improvement of uveitis defined as a reduction of intraocular inflammation according to Standardized Uveitis Nomenclature criteria. However, we observed a different response to treatment between the uveitis and the articular disease, as the latter did not respond as favorably as the former. Overall, the treatment was well tolerated by all patients and no ocular discomfort, ocular side effects, or allergic reactions were registered. JAK inhibitors may provide a new valuable treatment option in the therapeutic armamentarium for patients affected with JIA-associated uveitis, particularly in those refractory cases that are not adequately responding to conventional or biologic DMARDs.Key Points• A subset of patients with JIA uveitis either remain unresponsive or experience loss of efficacy• JAK inhibitors may provide a new valuable treatment option in JIA patients with uveitis• The safety profile was good with no occurrence of systemic side effects.

JAK inhibitors in refractory juvenile idiopathic arthritis-associated uveitis.

The diagnosis of Horner's syndrome (HS) can be difficult, as patients rarely present with the classic triad of ptosis, miosis, and anhydrosis. Frequently, there are no associated symptoms to help determine or localise the underlying pathology. The onset of anisocoria may also be uncertain, with many cases referred after incidental discovery on routine optometric assessment. Although the textbooks discuss the use of cocaine, apraclonidine, and hydroxyamphetamine to diagnose and localise HS, in addition to reported false positive and negative results, these pharmacological agents are rarely available during acute assessment or in general ophthalmic departments. Typically, a week is required between using cocaine or apraclonidine for diagnosis and localisation of HS with hydroxyamphetamine, leaving the clinician with the decision of which investigations to request and with what urgency. Modern imaging modalities have advanced significantly and become more readily available since many of the established management algorithms were written. We thus propose a practical and safe combined clinical and radiological diagnostic protocol for HS that can be applied in most clinical settings.

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Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common inflammatory joint condition of childhood and represents seven JIA subtypes characterised by distinct clinical and laboratory variables. Genetic and environmental factors are known to influence JIA, although many unanswered questions remain. Measurement of health outcomes in JIA is imperative for both clinical practice and research. Patient-reported outcomes present particular challenges in paediatric rheumatology in view of the importance of collecting reports from both the child/young person and the parent. Another challenge is the need for continuity of outcome measurement across the paediatric-adult interface during the process of transition in terms of both measurement tools and the mechanisms in the system to facilitate tracking of the young person into adult care. Finally, the need for adults with JIA to be seen as a distinct group in adult rheumatology practice is important for both service provision and outcome research.

Update on the epidemiology, risk factors and disease outcomes of juvenile idiopathic arthritis.

Background In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians’ experience. Consequently, treatment practices differ widely, within and between nations. Objectives To provide recommendations for the diagnosis and treatment of JIA-associated uveitis. Methods Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of nine experienced paediatric rheumatologists and three experts in ophthalmology from Europe. Recommendations derived from a validated systematic literature review were evaluated by an Expert Committee and subsequently discussed at two consensus meetings using nominal group techniques. Recommendations were accepted if >80% agreement was reached (including all three ophthalmologists). Results In total, 22 recommendations were accepted (with >80% agreement among experts): 3 on diagnosis, 5 on disease activity measurements, 12 on treatment and 2 on future recommendations. Conclusions The SHARE initiative aims to identify best practices for treatment of patients suffering from JIA-associated uveitis. Within this remit, recommendations for the diagnosis and treatment of JIA-associated uveitis have been formulated by an evidence-informed consensus process to suggest a standard of care for JIA-associated uveitis patients throughout Europe.

https://ard.bmj.com/content/annrheumdis/77/8/1107.full.pdf

Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative

Background The yield of imaging in Horner syndrome has been explored only in children This study evaluates the yield of imaging in adults Methods This was a retrospective cohort study of 52 patients with Horner syndrome examined in 2 neuro-ophthalmology hospital clinics Patients were divided into 3 groups according to the ability to determine the etiology at the time of the first neuro-ophthalmology consultation: group I, etiology of Horner syndrome known at the initial neuro-ophthalmologic examination; group II, etiology of Horner syndrome not known at the initial neuro-ophthalmologic examination, but sufficient information obtained to allow targeted imaging; and group III, etiology of Horner syndrome not known at the initial neuro-ophthalmologic examination, and sufficient information not obtained to allow targeted imaging The yield of investigation and the frequency of the different etiologies were evaluated Results In 32 (62%) patients, the etiology was already known at the initial neuro-ophthalmologic examination (group I) The most prevalent etiology was surgical trauma In 11 (21%) patients, a targeted imaging workup was possible, revealing an etiology in 7 patients (group II) Carotid dissection and cavernous sinus mass were the most common etiologies In 9 (17%) patients, a nontargeted imaging evaluation was necessary, revealing an etiology in only 1 patient, who had a previously undetected thyroid malignancy (group III) Conclusions The etiology of Horner syndrome is usually known at the time of initial presentation to a neuro-ophthalmologist When the etiology is not known and clinical information permits a targeted imaging evaluation, an etiology can usually be determined, most commonly a cervical carotid artery dissection or a cavernous sinus mass When the etiology is not known and clinical information is insufficient to allow a targeted imaging evaluation, an etiology is rarely discovered Even so, nontargeted imaging is warranted because life-threatening lesions, such as thyroid malignancies, may rarely be detected

Diagnostic value of imaging in horner syndrome in adults.

Red desaturation is a subjective, sensitive test for patients with optic nerve disease. There is no quantitative clinical test to measure the severity of color desaturation. The current study introduces a simple PowerPoint presentation for quantification of red desaturation in patients with optic neuritis. A Microsoft Office PowerPoint presentation was designed. A red square is shown on each half of the screen. The degree of desaturation on one side is increased in each successive slide. The patient holds a black cardboard divider between his/her eyes, such that each eye can see one-half of the screen. The patient uses the mouse to forward through the slides, thereby gradually increasing the desaturation of the red square presented to the normal eye until it matches the color of the red square seen with the tested eye. Desaturation is graded by the number of slide changes needed until subjective equal color between the eyes is perceived. The test was presented to normal volunteers and to patients with optic neuritis. The difference in saturation levels between the right and the left eyes in patients with optic neuritis vs controls was compared. A total of 83 patients were included. Patients with optic neuritis had significantly increased desaturation compared to controls (6.17 ± 3.3 vs 0.24 ± 2.8 respectively; p < 0.0001). This test is easy to create and efficiently quantifies the severity of red desaturation.

A simple computer program to quantify red desaturation in patients with optic neuritis.

The COVID-19 outbreak has led to the rapid development and administration of the COVID-19 vaccines worldwide. Data about the immunogenicity and adverse effects of the vaccine on patients with systemic autoimmune rheumatic diseases (SARDs) is emerging.To evaluate Pfizer/BioNTech (BNT162b2) mRNA-based vaccine second-dose immunogenicity and safety, and the relation between them, in patients with SARDs.A total of one hundred forty tow adults who received two doses of the BNT162b2 vaccine were included in the study. The SARDs group included Ninety-nine patients and the control group (forty-three participants) comprised a mixture of healthy participants and patients who were seen at the rheumatology clinic for non-SARDs. Anti-SARS-CoV-2 IgG antibodies against the Spike protein were evaluated using a SARS-CoV-2 IgG immunoassay. A level of > 150 AU/mL was considered positive. An adverse effects questionnaire was given to the participants upon their first visit to the clinic after their BNT162b2 vaccination.Of the 142 participants, 116 were seropositive (81.7%) and 26 (18.3%) were seronegative. Of the seronegative participants, 96.2% were SARDs patients. The proportion of seropositivity in the SARDs patients treated with any immunosuppressant was significantly lower (69.9%) compared to the control group and SARDs patients not receiving immunosuppressants (96.8%). A significant negative correlation between seronegativity and treatment with rituximab, mycophenolate mofetil (MMF), and prednisone was found in the SARDs group (p = 0.004, 0.044, 0.007 respectively). No fever was observed following the BNT162b2 vaccine in seronegative patients, and the frequency of musculoskeletal adverse effects upon the second dose of the BNT162b2 vaccine was significantly higher in seropositive compared to seronegative patients and in the control group compared to the SARDs patients (p = 0.045, p = 0.02 respectively).A decline in the immunogenicity to the second dose of BNT162b2 mRNA is seen in patients with SARDs, especially in patients treated with rituximab, MMF, and prednisone. Adverse effects of the vaccine including fever and musculoskeletal symptoms might be a signal for the acquisition of immunity in those patients.• BNT162b2 mRNA vaccine is less immunogenic in SARDs patients compared to the control group. • Rituximab, prednisone, and mycophenolate mofetil significantly reduced immunogenicity to the vaccine. • There is a correlation between immunogenicity and adverse effects of the vaccine. 

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Immunogenicity and safety of the mRNA-based BNT162b2 vaccine in systemic autoimmune rheumatic diseases patients

https://link.springer.com/content/pdf/10.1007/s10067-022-06412-8.pdf

Raz Gepstein

Papers

Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative

Diagnostic value of imaging in horner syndrome in adults.

A simple computer program to quantify red desaturation in patients with optic neuritis.

Immunogenicity and safety of the mRNA-based BNT162b2 vaccine in systemic autoimmune rheumatic diseases patients

Correction to: Immunogenicity and safety of the mRNA-based BNT162b2 vaccine in systemic autoimmune rheumatic diseases patients