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International Journal of Molecular... May 2023Behcet's disease (BD) is a chronic and recurrent systemic vasculitis involving almost all organs and tissues. Intestinal BD is defined as BD with predominant... (Review)
Review
Behcet's disease (BD) is a chronic and recurrent systemic vasculitis involving almost all organs and tissues. Intestinal BD is defined as BD with predominant gastrointestinal involvement, presenting severe complications such as massive gastrointestinal hemorrhage, perforation, and obstruction in some cases. To some extent, intestinal BD is classified as a member of inflammatory bowel disease (IBD), as it has a lot in common with classical IBD including Crohn's disease (CD) and ulcerative colitis (UC). Certainly, the underlying pathogenesis is not the same and dysregulation of immune function is believed to be one of the main pathogeneses in intestinal BD, although the etiology has not been clear up to now. Biological agents are an emerging category of pharmaceuticals for various diseases, including inflammatory diseases and cancers, in recent decades. Based on the deep understanding of the immune mechanism of intestinal BD, biological agents targeting potential pathogenic cells, cytokines and pathways are optimized options. Recently, the adoption of biological agents such as anti-tumor necrosis factor agents has allowed for the effective treatment of patients with refractory intestinal BD who show poor response to conventional medications and are faced with the risk of surgical treatment. In this review, we have tried to summarize the immune mechanism and present potential biological agents of intestinal BD.
Topics: Humans; Biological Factors; Behcet Syndrome; Intestines; Inflammatory Bowel Diseases; Crohn Disease
PubMed: 37175882
DOI: 10.3390/ijms24098176 -
Frontiers in Immunology 2023Non-infectious uveitis is a complex disease characterized by intraocular inflammation of the uveal area and the leading cause of vision impairment and blindness in young...
PURPOSE
Non-infectious uveitis is a complex disease characterized by intraocular inflammation of the uveal area and the leading cause of vision impairment and blindness in young people globally. However, what triggers inflammation and contributes to its recurrence remains unclear. The complement system has been linked to various immunological and inflammatory conditions. In the present study, we have systematically evaluated the role of the alternative complement pathway in the pathogenesis of non-infectious uveitis.
METHODOLOGY
Quantitative PCR was done in the peripheral leukocytes to study the expression of genes and regulatory miRNA in both anterior and posterior uveitis (n=28 in each category). Multiplex ELISA was performed to measure alternative pathway complement components, such as C3b, factor B, and CFH, and aqueous humor of infectious and non-infectious uveitis patients and non-inflammatory controls (n=10 each). Western blotting was done to validate the ELISA findings in a subset of patients and controls.
RESULTS
Downregulation of and mRNA in the peripheral blood was shown by quantitative PCR in the group of anterior uveiits (AU), while the opposite result was found in the group of posterior uveitis (PU). ELISA levels of C3b and CFH proteins were significantly higher in aqueous humor of infectious and non-infectious uveitis (*p = 0.03 and **p = 0.0007 respectively) as compared to the control group. Western blotting further validated (VitH) the activation of the complement cascade in the aqueous (AH) and vitreous humor of patients with non-infectious uveitis, with an increased level of C3b (n=6) and CFH (n=4) in aqueous humor. C3b level was significantly increased while CFH was reduced relative to controls in the vitreous humor (VitH) of posterior uveitis patients compared to controls (n=27 in each category). A C3b to CFH ratio was computed to assess the regulation of complement activation and this index was several folds higher in both anterior and posterior uveitis (n=10 each). The expression of miRNA-hsa-miR-146a and miRNA-hsa-miR-155-5p that regulates CFH was downregulated and nicely correlated with the increased complement proteins in both anterior and posterior uveitis (n=10 each).
CONCLUSION
Our results demonstrate a clear role of CFH and the activation of the alternative complement pathway in the pathogenesis of non-infectious uveitis; however, its therapeutic potential warrants further investigations.
Topics: Humans; Adolescent; Complement Pathway, Alternative; Uveitis; Uveitis, Posterior; MicroRNAs; Inflammation
PubMed: 38187376
DOI: 10.3389/fimmu.2023.1222998 -
Turkish Journal of Medical Sciences Aug 2021The aim of this study was to assess the nutritional status and risk factors for malnutrition in Behçet’s disease (BD) and neuro-Behçet’s disease (NBD) patients.
BACKGROUND/AIM
The aim of this study was to assess the nutritional status and risk factors for malnutrition in Behçet’s disease (BD) and neuro-Behçet’s disease (NBD) patients.
MATERIALS AND METHODS
Medical recordings of 74 patients with BD without neurological involvement (BDWoNI), 72 patients with NBD, and 50 patients with other diseases (carpal tunnel syndrome or lumbar discopathy) were analyzed retrospectively. The serum analyses were performed in the inactive period of disease. Chronic malnutrition was defined as low levels of serum albumin (<3.5 g/dL) with normal sedimentation rate and normal serum CRP levels.
RESULTS
Six (8.3%) of the patients in NBD group, 1 (1.4%) of the patients in BDWoNI group, and none of the patients in control group had chronic malnutrition (p = 0.029). Malnutrition rate was higher in NBD than control group (p = 0.036). The mean expanded disability status scale score was 2.92 ± 3.35 (range: 0–8) in patients with malnutrition and 1.44 ± 1.76 (range: 0–9) in patients without malnutrition in NBD group (p = 0.457). The ratio of patients having a progressive disease course was 33.3% and 7.6% in patients with and without malnutrition in NBD group, respectively. The median value of the duration of neurological involvement was 2 years (0–16) in patients with malnutrition and 6.5 years (0–18) in patients without malnutrition in NBD group (p = 0.046). There was no association between malnutrition and medications, disability scores, functional system involvement or findings on brain MRI.
CONCLUSION
Malnutrition was higher in patients with NBD. Higher disability level and progressive disease course may be risk factors for malnutrition in NBD. Malnutrition may be seen more frequently in earlier phases of neurological involvement.
Topics: Adult; Behcet Syndrome; Disease Progression; Humans; Magnetic Resonance Imaging; Malnutrition; Middle Aged; Nutritional Status; Retrospective Studies
PubMed: 33600096
DOI: 10.3906/sag-2009-235 -
Acta Ophthalmologica Feb 2022To evaluate the clinical course of idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and the efficacy and safety of treatment options during...
PURPOSE
To evaluate the clinical course of idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and the efficacy and safety of treatment options during pregnancy.
METHODS
Patients with MFC or PIC and a pregnancy in 2011-2019 from two academic centres were enrolled. For the most recent pregnancy, data on best-corrected visual acuity (BCVA) before and after pregnancy, relapse rate in pregnancy and postpartum period and obstetric, maternal and neonatal outcomes were collected. Treatment regimens consisted of a wait-and-see regime and an immunosuppressive treatment regime with systemic corticosteroids and/or azathioprine, both combined with intravitreal antivascular endothelial growth factor injections when indicated.
RESULTS
Sixteen women (26 affected eyes) were included. Median Snellen BCVA was 20/19 before pregnancy and 20/18 after delivery. In seven pregnancies a wait-and-see regime and in nine pregnancies an immunosuppressive treatment regime was carried out. Fourteen intravitreal anti-VEGF injections were given in six pregnancies. The relapse rate during pregnancy was 44% and in the postpartum period 31%. Maternal/obstetrical and fetal complications occurred in 31% and 13% of the pregnancies, respectively. Fifteen healthy children were born and one pregnancy ended in a stillbirth in a patient with a complicated obstetrical history. One patient treated with azathioprine developed intrahepatic cholestasis of pregnancy (ICP).
CONCLUSIONS
Among women with MFC and PIC BCVA remained stable during pregnancy despite a relapse rate of 44% in pregnancy. No major maternal, obstetric and fetal complications occurred in pregnant patients treated with systemic corticosteroids, azathioprine or intravitreal anti-VEGF injections, though one patient developed ICP while treated with azathioprine.
Topics: Angiogenesis Inhibitors; Choroiditis; Female; Fluorescein Angiography; Fundus Oculi; Humans; Immunosuppressive Agents; Intravitreal Injections; Pregnancy; Pregnancy Complications; Prognosis; Receptors, Vascular Endothelial Growth Factor; Retrospective Studies; Tomography, Optical Coherence; Visual Acuity; White Dot Syndromes
PubMed: 34009733
DOI: 10.1111/aos.14898 -
Acta Ophthalmologica Dec 2020To evaluate the efficacy of corticosteroid-sparing immunomodulatory therapy (IMT) in patients with recurrent and/or sight-threatening central multifocal choroiditis...
PURPOSE
To evaluate the efficacy of corticosteroid-sparing immunomodulatory therapy (IMT) in patients with recurrent and/or sight-threatening central multifocal choroiditis (MFC).
METHODS
This was a retrospective cohort study in a tertiary uveitis centre including all patients with MFC who have been treated with IMT for at least 12 months. Clinical data and imaging results were collected regarding the period prior to the start of IMT and at 3, 6, 12 and - where available - 24 months after the start of IMT. Main outcome measure was the number of annual recurrences of choroiditis with or without active choroidal neovascularization before and after the start of IMT. Secondary outcomes were the percentage of patients with (steroid-free) remission and the median time between the start of IMT and (steroid-free) remission.
RESULTS
Thirty-two patients (39 eyes) were included. At the start of IMT, none of the patients were in (steroid-free) remission. At 24 months, the probability of achieving remission and steroid-free remission was 88,5% and 50%, respectively. The median time to achieve remission and steroid-free remission was 21 and 83 weeks, respectively. In 17 patients (20 eyes) with available clinical data and imaging results for ≥ 12 months prior to the start of IMT, the mean number of recurrences/year decreased significantly from 1.40 ± 0.81 at baseline to 0.49 ± 0.47 (p = 0.001) after the start of IMT.
CONCLUSIONS
Preventive therapy with IMT should be considered in patients with recurrent and/or sight-threatening MFC to decrease the number of recurrences/year and to increase the prospects of achieving either remission or steroid-free remission.
Topics: Adolescent; Adult; Female; Follow-Up Studies; Glucocorticoids; Humans; Immunomodulation; Male; Middle Aged; Multifocal Choroiditis; Recurrence; Remission Induction; Retrospective Studies; Visual Acuity; Young Adult
PubMed: 32410393
DOI: 10.1111/aos.14473 -
Journal of B.U.ON. : Official Journal... 2020
Topics: Behcet Syndrome; Breast Neoplasms; Female; Humans
PubMed: 33099967
DOI: No ID Found -
La Tunisie Medicale Feb 2023Infectious uveitis (IU), one of the main causes of ocular inflammation in Tunisia, has various etiologies and clinical presentations. (Observational Study)
Observational Study
INTRODUCTION
Infectious uveitis (IU), one of the main causes of ocular inflammation in Tunisia, has various etiologies and clinical presentations.
AIM
To report the causes and clinical features of IU in a Tunisian population.
METHODS
Patients with IU referred to our department from February 2019 to January 2022 were included in a retrospective observational study. Ophthalmic examination and appropriate ancillary tests were performed.
RESULTS
Eighty eyes of 63 patients were included (37 female and 26 male). The mean age was 45 years. IU was unilateral in 43 cases, bilateral in 17, and unilateral alternating in three cases. Panuveitis was the most common anatomic location (35% of cases) followed by posterior (31%), anterior (18 eyes), intermediate (6 eyes) and anterior and intermediate uveitis (3 eyes). Non granulomatous uveitis (55%) occurred more frequently than granulomatous uveitis (45%). Etiologies were dominated by bacterial infections (29 patients; 46%), mainly tuberculosis (23 patients), followed by viruses (19 patients), mainly Herpes simplex virus. Fungal infection was found in one patient, while parasites accounted for 14 cases, mostly toxoplasmosis. Mean initial diagnostic delay was 16 months. The most common complications included occlusive retinal vasculitis (19 eyes), cataract (17 eyes), cystoid macular edema (17 eyes) and papilledema (15 eyes).
CONCLUSION
This study highlighted the causes and clinical features of IU in our setting. Tuberculosis remains one of its most frequent etiologies in Tunisia. However, a larger multicenter national study would allow a more precise evaluation.
Topics: Humans; Female; Male; Middle Aged; Delayed Diagnosis; Uveitis; Eye Infections, Bacterial; Panuveitis; Cataract
PubMed: 37682270
DOI: No ID Found -
Retinal Cases & Brief Reports Jul 2022To describe a novel clinical and imaging finding in patients with tubercular posterior uveitis.
PURPOSE
To describe a novel clinical and imaging finding in patients with tubercular posterior uveitis.
METHODS
A retrospective review of 3 cases presented at a tertiary referral eye centre in North India was performed between June 2016 and March 2019. All the patients had received an initial diagnosis of noninfective etiologies (sympathetic ophthalmia, necrotizing scleritis, and lymphoma). Fundus photography, fluorescein angiography, fundus autofluorescence, and enhanced-depth imaging optical coherence tomography were reviewed.
RESULTS
Three patients (all Asian Indian women: aged 18, 49, and 52 years) diagnosed with panuveitis were investigated for various etiologies based on the initial clinical suspicion. During the course of therapy, all the patients developed peripheral yellow subretinal pigment epithelium deposits (YSRPE) which appeared hypoautofluorescent on fundus autofluorescence and initially hypofluorescent with late hyperfluorescence on fluorescein angiography. The patients were subjected to detailed systemic evaluation and laboratory tests. All the patients showed acid-fast bacilli on invasive tissue biopsies. After initiation of antitubercular therapy, the lesions resolved in all eyes.
CONCLUSION
Yellow subretinal pigment epithelium deposits represent a novel and important diagnostic sign of tubercular posterior uveitis.
Topics: Epithelium; Female; Fluorescein Angiography; Humans; Retrospective Studies; Tomography, Optical Coherence; Tuberculosis, Ocular; Uveitis, Posterior
PubMed: 32541431
DOI: 10.1097/ICB.0000000000001016 -
BMJ Case Reports Mar 2022This case discusses a 10-year-old boy who presented in significant respiratory distress with cardiac tamponade with associated gross ascites and hepatomegaly, requiring...
This case discusses a 10-year-old boy who presented in significant respiratory distress with cardiac tamponade with associated gross ascites and hepatomegaly, requiring urgent transfer for pericardiocentesis. On further investigation, he was found to have multiple pulmonary emboli and evidence of panserositis. An underlying rheumatological cause was suspected in the absence of any evidence of infection or malignancy, and blood tests were positive for anti-double stranded DNA, anticardiolipin and antiglycoprotein antibodies as well as HLA B51. In his medical history, he has previously had mouth ulcers, chronic anaemia of undetermined cause and erythema multiforme. These symptoms, along with clinical presentation, mean a diagnosis of Behcet's disease and associated antiphospholipid syndrome was felt to be most likely. Anticoagulation therapy was commenced for treatment of the emboli, and colchicine was started for management of Behcet's disease. The patient was discharged clinically well from the hospital and continues under specialist rheumatological and haematological follow-up.
Topics: Behcet Syndrome; Child; Colchicine; HLA-B51 Antigen; Humans; Male; Oral Ulcer
PubMed: 35264387
DOI: 10.1136/bcr-2021-248134 -
BMJ Case Reports May 2022Intravesical Bacillus Calmette-Guérin (BCG) administration was used to treat bladder carcinoma in a woman in her 60s. Severe bilateral non-granulomatous anterior...
Intravesical Bacillus Calmette-Guérin (BCG) administration was used to treat bladder carcinoma in a woman in her 60s. Severe bilateral non-granulomatous anterior uveitis and gross papillitis developed subsequently. The severe BCG-induced bilateral uveitis and papillitis were treated with high dose oral corticosteroids, with topical steroids and cycloplegics. Resolution of her ocular symptoms and signs eventuated. On lumbar puncture, no evidence of systemic spread of the BCG was found. Visual acuity returned to 6/9 in each eye with subsequent resolution of papillitis. Repeat cystoscopy demonstrated no evidence of recurrent bladder tumour.Hypersensitivity reactions are well recognised with While both hypersensitivity reactions and dissemination of BCG throughout the body have been previously documented, the literature demonstrates that this case is the first example in which papillitis and bilateral uveitis were the prominent ophthalmological features.
Topics: Administration, Intravesical; BCG Vaccine; Female; Humans; Immunotherapy; Mycobacterium bovis; Papilledema; Urinary Bladder Neoplasms; Uveitis, Anterior
PubMed: 35606040
DOI: 10.1136/bcr-2021-247578