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Clinical Reviews in Allergy & Immunology Oct 2021Behçet's disease (BD) is a chronic, vasculitic disorder affecting all sizes of vessels. The disease rarely onsets at childhood and an early diagnosis is often... (Review)
Review
Behçet's disease (BD) is a chronic, vasculitic disorder affecting all sizes of vessels. The disease rarely onsets at childhood and an early diagnosis is often challenging. Oral ulceration and fever of unknown cause are common initial manifestations that might confuse other inflammatory disorders. The clinical manifestation pattern in pediatric BD is heterogeneous and varies in different genders, ethnicities, and geographic regions. There are also some differences in clinical presentations and prognosis between pediatric and adult BD. The disease also affects children at an extremely young age with mostly benign outcomes compared with that in older children. A limited number of studies reported issues about pediatric BD, let alone studies of children's treatments. Currently, the recommendation of the treatment in pediatric BD is according to the guideline of adult BD. The heterogeneity of clinical features makes the treatment more complicated. The main goal of the treatment is to control the inflammatory process and prevent recurrences. We will discourse the definition, epidemiology, clinical features, diagnosis, and treatment of pediatric BD in this review.
Topics: Behcet Syndrome; Child; Humans; Prognosis
PubMed: 32767171
DOI: 10.1007/s12016-020-08809-2 -
The Pan African Medical Journal 2017Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of...
Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of ophthalmology. We conducted a retrospective data collection from medical records of 20 patients treated at the military hospital in Laayoune. All patients underwent complete ophthalmological examination and fluorescein angiography if necessary. OCT exam was performed in two patients. Ten patients had anterior uveitis, complicated in one case by ocular hypertonia; two patients had intermediate uveitis; eight patients had posterior segment involvement complicated in one case by intravitreal hemorrhage. Behcet's Disease (BD) is an systemic idiopathic inflammatory disease currently classified within primary non-necrotizing vasculitis. Ocular involvement is common and severe in Behçet's disease, with the potential to compromise the visual prognosis. Behcet's disease is common in Morocco. It can compromise patient's visual prognosis making the collaboration between ophthalmologists and internists particularly important.
Topics: Adult; Behcet Syndrome; Eye Diseases; Female; Fluorescein Angiography; Hospitals, Military; Humans; Male; Morocco; Prognosis; Retrospective Studies; Severity of Illness Index; Uveitis, Anterior
PubMed: 28690751
DOI: 10.11604/pamj.2017.26.237.1175 -
Ocular Immunology and Inflammation May 2021: COVID-19 vaccination has been accompanied by reports of inflammatory events. This report details a case of panuveitis following vaccination for COVID-19 Case.: A 43...
: COVID-19 vaccination has been accompanied by reports of inflammatory events. This report details a case of panuveitis following vaccination for COVID-19 Case.: A 43 year old female developed panuveitis with decreased vision three days after her second dose of Pfizer-Biontech mRNA vaccine. The choroid was significantly thickened and there was anterior chamber and vitreous inflammation. Shortly after onset of ocular symptoms she was also found have an asymptomatic COVID-19 infection. Treatment with oral and topical corticosteroids resulted in improvement in the panuveitis, with a mild recurrence after the initial attempt to taper these drugs.: This report demonstrates a likely occurrence of vaccine-related panuveitis secondary to the Pfizer-Biotech mRNA vaccine for COVID-19.
Topics: Adult; Anterior Chamber; COVID-19; COVID-19 Vaccines; Female; Humans; Panuveitis; SARS-CoV-2; Tomography, Optical Coherence; Vaccination
PubMed: 34213988
DOI: 10.1080/09273948.2021.1949478 -
Current Opinion in Rheumatology Jan 2016Current trends in the management of Behçet's syndrome will be reviewed in this article. (Review)
Review
PURPOSE OF REVIEW
Current trends in the management of Behçet's syndrome will be reviewed in this article.
RECENT FINDINGS
Biologic agents have gained increasing importance over the years in the management of Behçet's syndrome. Long-term results of observational studies have shown that anti-tumor necrosis factor agents may be effective in Behçet's syndrome patients with refractory eye involvement. Case series reporting about use of anti-tumor necrosis factor agents in vascular and gastrointestinal involvement have also shown good results. Caution is required for infectious complications with these agents. Apremilast is an immunomodulatory agent that works through phosphodiesterase 4 inhibition. A randomized controlled trial has shown that it is effective for the management of oral and genital ulcers and is generally well tolerated.
SUMMARY
The outcome of Behçet's syndrome with major organ involvement has improved with more effective management strategies, especially with the use of biologic agents in severe cases. Controlled trials are needed to guide physicians in making treatment decisions.
Topics: Behcet Syndrome; Humans
PubMed: 26555450
DOI: 10.1097/BOR.0000000000000231 -
Revue Medicale de Liege Jan 2024Behçet disease is a chronic multisystemic inflammatory condition. The ocular disease mainly manifests as a bilateral, non-granulomatous, posterior uveitis or... (Review)
Review
Behçet disease is a chronic multisystemic inflammatory condition. The ocular disease mainly manifests as a bilateral, non-granulomatous, posterior uveitis or panuveitis, associated with occlusive retinal vasculitis. Uveitis is frequent in Behçet disease, it may be severe, and visually threatening. Early diagnosis and aggressive treatment are mandatory to preserve visual function. Therefore, the goal of this review is to describe the new diagnosis tools and therapeutic guidelines allowing for a significant improvement of the visual prognosis.
Topics: Humans; Behcet Syndrome; Uveitis; Retinal Vasculitis; Prognosis
PubMed: 38223970
DOI: No ID Found -
Ophthalmology Jun 2021To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis. (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis.
DESIGN
Open-label, multicenter, phase 3 extension study (VISUAL III).
PARTICIPANTS
Adults who had completed a randomized, placebo-controlled phase 3 parent trial (VISUAL I or II) without treatment failure (inactive uveitis) or who discontinued the study after meeting treatment failure criteria (active uveitis).
METHODS
Patients received subcutaneous adalimumab 40 mg every other week. Data were collected for ≤ 362 weeks. Adverse events (AEs) were recorded until 70 days after the last dose.
MAIN OUTCOME MEASURES
Long-term safety and quiescence; other efficacy variables included inflammatory lesions, anterior chamber cell and vitreous haze grade, macular edema, visual acuity, and dose of uveitis-related systemic corticosteroids.
RESULTS
At study entry, 67% of patients (283/424) showed active uveitis and 33% (141/424) showed inactive uveitis; 60 patients subsequently met exclusion criteria, and 364 were included in the intention-to-treat analysis. Efficacy variables were analyzed through week 150, when approximately 50% of patients (214/424) remained in the study. Patients showing quiescence increased from 34% (122/364) at week 0 to 85% (153/180) at week 150. Corticosteroid-free quiescence was achieved by 54% (66/123) and 89% (51/57) of patients with active or inactive uveitis at study entry. Mean daily dose of systemic corticosteroids was reduced from 9.4 ± 17.1 mg/day at week 0 (n = 359) to 1.5 ± 3.9 mg/day at week 150 (n = 181). The percentage of patients who achieved other efficacy variables increased over time for those with active uveitis at study entry and was maintained for those with inactive uveitis. The most frequently reported treatment-emergent AEs of special interest were infections (n = 275; 79 events/100 patient-years [PY]); AEs and serious AEs occurred at a rate of 396 events/100 PY and 15 events/100 PY, respectively.
CONCLUSIONS
Long-term treatment with adalimumab led to quiescence and reduced corticosteroid use for patients who entered VISUAL III with active uveitis and led to maintenance of quiescence for those with inactive uveitis. AEs were comparable with those reported in the parent trials and consistent with the known safety profile of adalimumab.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Injections, Subcutaneous; Male; Middle Aged; Panuveitis; Retrospective Studies; Time Factors; Treatment Outcome; Uveitis, Intermediate; Uveitis, Posterior; Visual Acuity; Young Adult
PubMed: 33157077
DOI: 10.1016/j.ophtha.2020.10.036 -
Klinische Monatsblatter Fur... May 2020Syphilis is a bacterial infectious disease transmitted by the spirochaete Treponema pallidum. The rate of infection has increased during the last decade. Ocular syphilis...
Syphilis is a bacterial infectious disease transmitted by the spirochaete Treponema pallidum. The rate of infection has increased during the last decade. Ocular syphilis is an underestimated presentation of the disease, and it is increased in HIV-positive patients. Even though every part of the eye may be affected, syphilis most commonly occurs with posterior or panuveitis. A distinctive pattern is acute syphilitic placoid chorioretinitis, with typical features in multimodal imaging. If syphilis is suspected, specific and nonspecific Treponema pallidum serological diagnostic tests are mandatory. Clinical outcome, or morphology and vision are commonly improved if antibiotic therapy (penicillin is the drug of choice) is instituted early. Additional corticosteroids are carefully applied according to individual need, and should be initiated after the start of antibiotics.
Topics: Anti-Bacterial Agents; Chorioretinitis; Endophthalmitis; Eye Infections, Bacterial; Humans; Panuveitis; Syphilis
PubMed: 32434244
DOI: 10.1055/a-1126-8970 -
Retina (Philadelphia, Pa.) Nov 2022To characterize optical coherence tomography features in patients with idiopathic intermediate, posterior, or panuveitis.
PURPOSE
To characterize optical coherence tomography features in patients with idiopathic intermediate, posterior, or panuveitis.
METHODS
This is a retrospective case series of all consecutive cases of idiopathic intermediate, posterior, or panuveitis at four tertiary care centres between 2010 and 2021.
RESULTS
A total of 94 eyes (55 patients) were followed for an average duration of 29.8 (SD 21) months. The median central macular thickness was 284 µ m at baseline and 267 µ m at last follow-up. At baseline and last follow-up, respectively, 24% and 20% of uveitic eyes had intraretinal fluid, 12% and 1% subretinal fluid, and 43% and 54% epiretinal membrane. In addition, ellipsoid zone abnormalities on en-face were noted in 34% and 19% of cases at baseline and last follow-up, respectively. The baseline median visual acuity was significantly lower among cases with ellipsoid zone en-face slab abnormalities compared with those without (0.2 logarithm of minimum angle of resolution [interquartile range: 0-0.6] vs. 0.1 logarithm of minimum angle of resolution [interquartile range: 0-0.3], P = 0.0051).
CONCLUSION
With initiation of treatment, the central macular thickness, intraretinal fluid, subretinal fluid, and ellipsoid zone en-face abnormalities improved over time, whereas the number of cases with epiretinal membrane increased among eyes with idiopathic intermediate, posterior, and panuveitis. Presence of ellipsoid zone en-face abnormalities at presentation may be associated with worse visual acuity.
Topics: Humans; Tomography, Optical Coherence; Epiretinal Membrane; Retrospective Studies; Panuveitis; Visual Acuity; Papilledema
PubMed: 36070567
DOI: 10.1097/IAE.0000000000003572 -
Ophthalmic Plastic and Reconstructive...Prophylactic enucleation of a ruptured globe with no light perception within 14 days of injury to prevent sympathetic ophthalmia (SO) has been an established dictum in... (Review)
Review
PURPOSE
Prophylactic enucleation of a ruptured globe with no light perception within 14 days of injury to prevent sympathetic ophthalmia (SO) has been an established dictum in academic teaching for more than 100 years. This treatment strategy was originally based on observation, speculation, and careful thought, but there was never any scientific proof. This review summarizes and updates the current state of our knowledge about globe rupture and SO, examines the origin and validity of the 14-day rule, and emphasizes the importance of trying to save the traumatized eye whenever possible.
METHODS
A comprehensive literature review of SO and globe rupture was performed.
RESULTS
SO is a rare disorder that may potentially occur following traumatic globe rupture as well as following a variety of other intraocular surgeries. Vitreoretinal surgery may be a more common cause than trauma according to some studies. SO may still occur despite having the eye removed within 14 days of the trauma. A variety of new medications including biologic agents are now available to treat SO with improved efficacy in suppressing the associated ocular inflammation and allowing retention of some useful vision. Removing the traumatized, blind eye may have other important psychological consequences associated with it that require consideration before eye removal is carried out. Retaining the blind, phthisical, disfigured eye avoids phantom vision and phantom pain associated with enucleation as well as providing a good platform to support and move an overlying prosthetic eye. Data on the occurrence of SO following evisceration and enucleation with and without predisposing factors confirms the exceedingly low risk.
CONCLUSION
Most civilian open globe injuries can be successfully repaired with modern, advanced microsurgical techniques currently available. Because of the exceedingly low risk of SO, even with the severity of open globe trauma during military conflicts being more devastating as a result of the blast and explosive injuries, today every attempt is made to primarily close the eye rather than primarily enucleate it, providing there is enough viable tissue to repair. The 14-day rule for eye removal after severe globe ruptures is not scientifically supported and does not always protect against SO, but the safe time period for prophylactic eye removal is not definitively known. In the exceptional cases where SO does occur, several new medications are now available that may help treat SO. We advocate saving the ruptured globe whenever possible and avoiding prophylactic enucleation to prevent the rare occurrence of SO. When an eye requires removal, evisceration is an acceptable alternative to enucleation in cases that do not harbor intraocular malignancy.
Topics: Eye Enucleation; Eye Evisceration; Eye Injuries, Penetrating; Humans; Ophthalmia, Sympathetic; Retrospective Studies; Rupture
PubMed: 34593714
DOI: 10.1097/IOP.0000000000002068 -
Ophthalmology Dec 2018In recent decades, the treatment paradigm for noninfectious intermediate uveitis, posterior uveitis, and panuveitis, a group of intraocular inflammatory diseases, has... (Review)
Review
In recent decades, the treatment paradigm for noninfectious intermediate uveitis, posterior uveitis, and panuveitis, a group of intraocular inflammatory diseases, has included systemic and local (periocular or intraocular) corticosteroids, biologics, and other steroid-sparing immunomodulatory therapy agents. Recently, an intravitreal formulation of sirolimus, an immunosuppressant that inhibits the mammalian target of rapamycin, a key regulator of cell growth in the immune system, was developed. On the basis of this mechanism and the local method of delivery, it was hypothesized that intravitreal sirolimus can improve ocular inflammation in patients with noninfectious intermediate uveitis, posterior uveitis, and panuveitis, with minimal systemic exposure and systemic adverse events (AEs). This review summarizes the pharmacokinetics, efficacy, and safety results of intravitreal sirolimus from 3 preclinical studies and 4 phase 1-3 clinical studies. Preclinical studies in rabbits showed that 22 to 220 μg intravitreal sirolimus results in sustained release of sirolimus in the vitreous for 2 months or more, with systemic concentrations below the threshold for systemic immunosuppression (approximately 8 ng/ml). Subsequently, 2 phase 1 studies (n = 50 and n = 30) established that intravitreal sirolimus improves ocular inflammation in humans. Further investigation in phase 2 and 3 studies (n = 24 and n = 347, respectively) suggested that 440 μg has the best benefit-to-risk profile. In the phase 3 study, the proportion of patients who showed complete resolution of ocular inflammation at month 5 was significantly higher in the 440-μg group than in the 44-μg group (22.8% vs. 10.3%; P = 0.025, Fisher exact test). In addition, 47 of 69 patients (68.1%) who were treated with systemic corticosteroids at baseline discontinued corticosteroid use at month 5. No sirolimus-related systemic AEs were reported in phase 1-3 studies. Collectively, these preclinical and clinical study data of intravitreal sirolimus support the therapeutic rationale of treating noninfectious uveitis with a local mammalian target of rapamycin inhibitor and suggest that 440 μg intravitreal sirolimus has the potential to be an effective and well-tolerated anti-inflammatory and corticosteroid-sparing treatment for noninfectious intermediate uveitis, posterior uveitis, and panuveitis.
Topics: Animals; Clinical Trials as Topic; Drug Evaluation, Preclinical; Humans; Immunosuppressive Agents; Intravitreal Injections; Panuveitis; Sirolimus; Uveitis, Intermediate; Uveitis, Posterior
PubMed: 30060978
DOI: 10.1016/j.ophtha.2018.06.015