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Journal of Neuroinflammation May 2024The human gut microbiome (GM) is involved in inflammation and immune response regulation. Dysbiosis, an imbalance in this ecosystem, facilitates pathogenic invasion,...
BACKGROUND
The human gut microbiome (GM) is involved in inflammation and immune response regulation. Dysbiosis, an imbalance in this ecosystem, facilitates pathogenic invasion, disrupts immune equilibrium, and potentially triggers diseases including various human leucocyte antigen (HLA)-B27-associated autoinflammatory and autoimmune diseases such as inflammatory bowel disease (IBD) and spondyloarthropathy (SpA). This study assesses compositional and functional alterations of the GM in patients with HLA-B27-associated non-infectious anterior uveitis (AU) compared to healthy controls.
METHODS
The gut metagenomes of 20 patients with HLA-B27-associated non-infectious AU, 21 age- and sex-matched HLA-B27-negative controls, and 6 HLA-B27-positive healthy controls without a history of AU were sequenced using the Illumina NovaSeq 6000 platform for whole metagenome shotgun sequencing. To identify taxonomic and functional features with significantly different relative abundances between groups and to identify associations with clinical metadata, the multivariate association by linear models (MaAsLin) R package was applied.
RESULTS
Significantly higher levels of the Eubacterium ramulus species were found in HLA-B27-negative controls (p = 0.0085, Mann-Whitney U-test). No significant differences in microbial composition were observed at all other taxonomic levels. Functionally, the lipid IV biosynthesis pathway was upregulated in patients (p < 0.0001, Mann-Whitney U-test). A subgroup analysis comparing patients with an active non-infectious AU to their age- and sex-matched HLA-B27-negative controls, showed an increase of the species Phocaeicola vulgatus in active AU (p = 0.0530, Mann-Whitney U-test). An additional analysis comparing AU patients to age- and sex-matched HLA-B27-positive controls, showed an increase of the species Bacteroides caccae in controls (p = 0.0022, Mann-Whitney U-test).
CONCLUSION
In our cohort, non-infectious AU development is associated with compositional and functional alterations of the GM. Further research is needed to assess the causality of these associations, offering potentially novel therapeutic strategies.
Topics: Humans; HLA-B27 Antigen; Female; Male; Gastrointestinal Microbiome; Middle Aged; Uveitis, Anterior; Adult; Case-Control Studies; Aged
PubMed: 38715051
DOI: 10.1186/s12974-024-03109-4 -
Medical Science Monitor : International... May 2024Behçet uveitis poses significant management challenges, owing to its intricate pathogenesis and the severe prognosis it harbors, frequently culminating in irreversible... (Review)
Review
Behçet uveitis poses significant management challenges, owing to its intricate pathogenesis and the severe prognosis it harbors, frequently culminating in irreversible visual impairment and an elevated risk of blindness. This review synthesizes contemporary insights into personalized immunosuppressive strategies for Behçet uveitis, emphasizing the necessity for a customized approach in recognition of the disease's heterogeneity and the variable responsiveness to treatment. This discourse elaborates on the application, efficacy, and safety profiles of traditional immunosuppressants, highlighting a paradigm shift toward integrative combination therapies aimed at diminishing reliance on glucocorticoids and mitigating their associated adverse effects. This thorough evaluation seeks to enlighten clinical practices and spearhead future investigations aimed at refining the management of Behçet uveitis, championing a personalized, multidisciplinary strategy to amplify therapeutic efficacy and enhance patient quality of life.
Topics: Humans; Behcet Syndrome; Uveitis; Immunosuppressive Agents; Precision Medicine; Quality of Life
PubMed: 38711247
DOI: 10.12659/MSM.943240 -
Rheumatology (Oxford, England) May 2024Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause...
INTRODUCTION
Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging.
OBJECTIVES
To evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis.
METHODS
Retrospective chart review of non-infectious PGU occurring in children before the age of 16 years recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023.
RESULTS
We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with juvenile idiopathic arthritis, and one with Vogt-Koyanagi-Harada disease. Median age at diagnosis was 9.8 years (range 7.2-12.5). The sex-ratio M/F was 0.52. The most common features of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%) particularly in cases of panuveitis. The most commonly used treatments were systemic corticosteroids (72%) and methotrexate (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years.
CONCLUSION
We report the largest cohort of PGU. PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management.
PubMed: 38710493
DOI: 10.1093/rheumatology/keae253 -
Cureus Apr 2024Behçet's disease (BD) is a vascular disorder affecting a variety of organ systems. It is an auto-immune disease with inflammatory vasculitis that is systemic in nature,...
Behçet's disease (BD) is a vascular disorder affecting a variety of organ systems. It is an auto-immune disease with inflammatory vasculitis that is systemic in nature, the exact etiology of which is unknown. Obliterative vasculitis, recurrent aphthous ulcers, mucocutaneous manifestations, recurrent genital ulcerations, and intraocular inflammation, especially chronic relapsing uveitis, are the characteristic features of BD. The case report presents a unique manifestation of BD in a 20-year-old Pakistani male who presents with a one-year-old history of viral encephalitis, after which he developed a blurring of vision. On examination, he had recurrent aphthous ulcers, recurrent ulcerations of genitalia, and a history of lesions of the skin. After making the patient undergo a cascade of investigations for evaluating and assessing the various signs and symptoms, a diagnosis of BD with bilateral panuveitis and a full-thickness macular hole (FTMH) in the right eye was established. Immuno-suppressants, steroids, and azathioprine were used as treatment options, following which the state of remission was attained.
PubMed: 38707073
DOI: 10.7759/cureus.57599 -
American Journal of Ophthalmology May 2024To ascertain whether the use of ultra-wide-field fluorescein angiography (UWFFA) at baseline visit alters the assessment of disease activity and localization, as well as...
PURPOSE
To ascertain whether the use of ultra-wide-field fluorescein angiography (UWFFA) at baseline visit alters the assessment of disease activity and localization, as well as the management of patients presenting to a tertiary uveitis clinic.
DESIGN
Retrospective comparison of diagnostic approaches.
METHODS
Baseline visits of 158 patients who presented to the Uveitis Clinic at the Byers Eye Institute at Stanford between 2017 and 2022 were evaluated by 3 uveitis-trained ophthalmologists (I.K., A.B., and H.G.). Each eye had undergone clinical examination along with ultra-wide-field fundus photography (UWFFP) (Optos Plc), spectral-domain optical coherence tomography (SD-OCT, Spectralis Heidelberg, Heidelberg Engineering) and UWFFA (Optos Plc) at the baseline visit. Investigators were asked to successively determine disease activity, localization of disease (anterior, posterior or both), and management decisions based on clinical examination and UWFFP and SD-OCT (Set 1) and Set 1 plus UWFFA (Set 2). The primary outcome was the percentage of eyes whose management changed based on the availability of UWFFA compared with Set 1.
RESULTS
The mean age of the patients was 46.9 ± 22.4 years (range, 7-96), and 91 (57.6%) were female. With Set 1 alone, 138 eyes (55.2%) were found to have active disease; localization was anterior in 58 eyes (42.0%), posterior in 53 eyes (38.4%), and anterior + posterior in 27 eyes (19.6%). With Set 2, 169 eyes of 107 patients had active anterior, posterior, or panuveitis. In comparison with Set 1, assessment with Set 2 identified additional 31 eyes (18.3%) with active disease (P = .006) and an additional 31 eyes (18.3%) having disease in both anterior + posterior segments (P < .001). Regarding the primary outcome, management was changed in 68 eyes (27.4%) in Set 2 compared with Set 1.
CONCLUSIONS
Baseline UWFFA may alter assessment of disease activity, localization, and management decisions compared with clinical examination with only UWFFP and SD-OCT for eyes with uveitis. Thus, UWFFA may be considered as an essential tool in the evaluation of patients with uveitis at the baseline visit.
PubMed: 38701875
DOI: 10.1016/j.ajo.2024.04.016 -
The Lancet. Haematology May 2024
Topics: Behcet Syndrome; Humans; Hemorrhage; Blood Coagulation
PubMed: 38697732
DOI: 10.1016/S2352-3026(24)00095-4 -
Journal de Medecine Vasculaire Apr 2024Behçet's disease is a systemic vasculitis characterized by recurrent bipolar aphtosis and ophthalmic disorders. Cardiac involvement is rarely reported and could be...
Behçet's disease is a systemic vasculitis characterized by recurrent bipolar aphtosis and ophthalmic disorders. Cardiac involvement is rarely reported and could be associated to poor prognosis. Intracardiac thrombosis is exceptional and represents a therapeutic issue. We report the case of a young man admitted in internal medicine department for management of prolonged fever and recurrent mouth ulcers.
Topics: Humans; Behcet Syndrome; Male; Recurrence; Adult; Treatment Outcome; Thrombosis; Heart Diseases; Anticoagulants
PubMed: 38697708
DOI: 10.1016/j.jdmv.2024.02.002 -
Journal Francais D'ophtalmologie Mar 2024To evaluate and compare characteristics, diagnosis, treatment, visual prognosis, and course between ocular sarcoidosis with or without uveitis in a population in...
OBJECTIVE
To evaluate and compare characteristics, diagnosis, treatment, visual prognosis, and course between ocular sarcoidosis with or without uveitis in a population in Southern France.
METHODS
We retrospectively analyzed data from patients with ocular sarcoidosis in a tertiary eye care center in Nice from January 2003 to December 2021. The inclusion criterion was biopsy-proven ocular sarcoidosis according to IWOS criteria as the first clinical manifestation of sarcoidosis.
RESULTS
A total of 25 patients were included. Twenty patients had uveitis (70% panuveitis, 20% intermediate uveitis, and 10% anterior uveitis) and five patients had non-uveitic ocular sarcoidosis (one patient with dacryoadenitis, one patient with orbital granuloma, two patients with palpebral granuloma, and one patient with episcleritis). Only the cases with uveitis had bilateral involvement (85% of cases). There was no significant difference in ethnicity, biopsy diagnosis, systemic manifestations, or treatment between the two groups. Final visual outcomes remained good for both groups, with 96% of patients with BCVA>20/50, with no significant difference. Patients with non-uveitic sarcoidosis experienced less recurrence on treatment (P=0.042) and more remission (P=0.038) than patients with uveitis. Eighty percent of patients with uveitis had at least three suggestive clinical intraocular signs meeting IWOS criteria.
CONCLUSION
In this population in Southern France, uveitis was the most common presentation of ocular sarcoidosis. The type of ocular sarcoidosis does not appear to be correlated with the type of systemic manifestations, use of systemic therapy, or visual prognosis, but patients with non-uveitic ocular sarcoidosis appear to have a better course with fewer recurrences on treatment and more remission than patients with uveitic ocular sarcoidosis.
PubMed: 38696861
DOI: 10.1016/j.jfo.2024.104153 -
Ocular Immunology and Inflammation May 2024To describe the efficacy and safety of adalimumab for the treatment of non-infectious uveitis (NIU) in four Uveitis Units from tertiary Spanish hospitals.
OBJECTIVE
To describe the efficacy and safety of adalimumab for the treatment of non-infectious uveitis (NIU) in four Uveitis Units from tertiary Spanish hospitals.
METHODS
Multicenter and retrospective clinical cohort study including all patients with NIU treated with adalimumab from January 2012 to October 2022 in four uveitis units was performed. Efficacy was measured with the number of relapses, ocular inflammation and reduction in immunosuppression and corticosteroid dosage before and after adalimumab use. We collected data regarding adverse effects and examined the immunogenicity of adalimumab.
RESULTS
One hundred and twenty-two patients (59% females), with a mean age of 48.6 years (SD = 14.8) accounting for 217 eyes were included. The majority (92.6%) were Caucasian. Uveitis analyzed were predominantly panuveitis (34.7%), bilateral (77.9%), acute (41.5%), and non-granulomatous (90%). Most of them were immune mediated (42.6%), and the main reason to initiate adalimumab was refractory disease (96.7%). The analysis was statistically significant due to the reduction in the number of immunosuppressive drugs as well as the dose of oral corticosteroids and the number of relapses during follow-up ( < 0.001). The decrease in ocular inflammation parameters and the improvement in visual acuity ( < 0.05) were also significant. There were no deaths due to the drug and only one reported case of serious infection. In total, 10.9% of 73 patients tested developed anti-adalimumab antibodies and 4.1% lupus-like.
CONCLUSIONS
We consider adalimumab as a leading drug in the treatment of NIU with high safety and efficacy.
PubMed: 38691840
DOI: 10.1080/09273948.2024.2344712 -
Frontiers in Immunology 2024Little is known about the relationship between the disease activity of Behçet disease (BD) and the incidence of inflammatory major organ events.
BACKGROUND
Little is known about the relationship between the disease activity of Behçet disease (BD) and the incidence of inflammatory major organ events.
OBJECTIVES
In this prospective registry study, we investigated the association between the Behçet Disease Current Activity Form (BDCAF) and incidence of inflammatory major organ events, defined as the inflammation of the ocular, central nervous, intestinal, and vascular systems in BD.
METHODS
We enrolled participants from Japanese multicenter prospective cohorts. The BDCAF was evaluated annually. BD-related symptoms, including inflammatory major organ events, were monitored. The association between BDCAF and inflammatory major organ events was analyzed by time-to-event analysis. An unsupervised clustering of the participants' BDCAF, therapeutic agents, and multiple serum cytokines was also performed to examine their association with inflammatory major organ events.
RESULTS
A total of 260 patients were included. The patients had a median BDCAF score of 2 [Interquartile range, 1-3] at the enrolment and remained disease active at 1- and 2-year follow-ups, indicating residual disease activity in BD. Patients with a BDCAF score of 0 had a longer inflammatory major organ event-free survival at 52 weeks than those with a score of 1 or higher (p=2.2 x 10). Clustering analysis revealed that patients who did not achieve remission despite treatment with tumor necrosis factor inhibitors had high serum inflammatory cytokine levels and incidences of inflammatory major organ events. Among the elevated cytokines, IL-6 was associated with inflammatory major organ events.
CONCLUSION
This study suggests that treatment strategies targeting overall disease activity and monitoring residual serum IL-6 may help prevent inflammatory major organ events in BD.
Topics: Behcet Syndrome; Humans; Male; Female; Registries; Interleukin-6; Adult; Prospective Studies; Incidence; Middle Aged; Inflammation; Biomarkers; Japan; Severity of Illness Index
PubMed: 38686380
DOI: 10.3389/fimmu.2024.1354969