ABSTRACT
BACKGROUND: Hodgkin's lymphoma (HL) is an extremely rare cause of ocular inflammation that is usually not considered in the typical workup of uveitis and other eye diseases. A few cases of ocular inflammation were reported previously showcasing HL with absence of typical symptoms of HL at presentation. Acknowledging the potential ocular inflammation associated with HL can prompt ophthalmologists to broaden their diagnostic approach and collaborate with internal medicine departments to investigate this rare yet significant etiology. CASE PRESENTATION: A 17-year-old Caucasian woman presenting unilateral panuveitis was later diagnosed with HL. The ocular findings were non-necrotizing scleritis, anterior uveitis, vitritis, white/yellowish chorioretinal lesions, papillitis and vasculitis. A left supra-clavicular lymph node biopsy confirmed the diagnosis of nodular sclerosing Hodgkin's lymphoma stage IIB. Other causes of uveitis were excluded. Chemotherapy led to remission of the disease and the ocular lesions became quiescent with persistent pigmented chorioretinal scars. CONCLUSIONS: Hodgkin's lymphoma should be considered in the differential diagnosis of diseases that can occasionally be revealed by unilateral ocular inflammation. A comprehensive, multidisciplinary approach is key to properly assessing such cases.
Subject(s)
Hodgkin Disease , Humans , Hodgkin Disease/diagnosis , Female , Adolescent , Diagnosis, Differential , Scleritis/diagnosis , Scleritis/etiology , Scleritis/drug therapy , Uveitis/diagnosis , Uveitis/drug therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiologyABSTRACT
Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18F-fluoro-2-deoxy-2-d-glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren's syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone for several weeks resulted in significant improvement. This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent a delayed diagnosis.
Subject(s)
Histiocytosis, Sinus , Panuveitis , Sjogren's Syndrome , Humans , Female , Adult , Histiocytosis, Sinus/complications , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/pathology , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Fluorodeoxyglucose F18 , Positron Emission Tomography Computed Tomography , Hypertrophy , Panuveitis/complications , Panuveitis/diagnosis , Panuveitis/drug therapyABSTRACT
PURPOSE: To evaluate features of infectious panuveitis associated with multiple pathogens detected by ocular fluid sampling. METHODS: Single-center, retrospective, consecutive case series of patients with aqueous/vitreous polymerase chain reaction testing with >1 positive result in a single sample from 2001 to 2021. RESULTS: Of 1,588 polymerase chain reaction samples, 28 (1.76%) were positive for two pathogens. Most common pathogens were cytomegalovirus (n = 16, 57.1%) and Epstein-Barr virus (n = 13, 46.4%), followed by varicella zoster virus (n = 8, 28.6%), Toxoplasma gondii (n = 6, 21.4%), herpes simplex virus 2 (n = 6, 21.4%), herpes simplex virus 1 (n = 6, 21.4%), and Toxocara (n = 1, 3.6%). Mean initial and final visual acuity (logarithm of the minimum angle of resolution) were 1.3 ± 0.9 (Snellen â¼20/400) and 1.3 ± 1.1 (Snellen â¼20/400), respectively. Cytomegalovirus-positive eyes (n = 16, 61.5%) had a mean final visual acuity of 0.94 ± 1.1 (Snellen â¼20/175), whereas cytomegalovirus-negative eyes (n = 10, 38%) had a final visual acuity of 1.82 ± 1.0 (Snellen â¼20/1,320) ( P < 0.05). Main clinical features included intraocular inflammation (100%), retinal whitening (84.6%), immunosuppression (65.4%), retinal hemorrhage (38.5%), and retinal detachment (34.6%). CONCLUSION: Cytomegalovirus or Epstein-Barr virus were common unique pathogens identified in multi-PCR-positive samples. Most patients with co-infection were immunosuppressed with a high rate of retinal detachment and poor final visual acuity. Cytomegalovirus-positive eyes had better visual outcomes compared with cytomegalovirus-negative eyes.
Subject(s)
Aqueous Humor , Eye Infections, Viral , Panuveitis , Polymerase Chain Reaction , Visual Acuity , Humans , Retrospective Studies , Male , Female , Panuveitis/diagnosis , Panuveitis/virology , Panuveitis/drug therapy , Middle Aged , Aqueous Humor/virology , Eye Infections, Viral/diagnosis , Eye Infections, Viral/virology , Adult , Aged , DNA, Viral/analysis , Vitreous Body/virology , Cytomegalovirus/genetics , Cytomegalovirus/isolation & purification , Young Adult , Toxoplasma/isolation & purification , Toxoplasma/geneticsABSTRACT
PURPOSE: To report the first series of Seasonal Hyperacute Pan Uveitis (SHAPU) from Bhutan. METHODS: We retrospectively analyzed the patients with clinically diagnosed SHAPU treated in the referral center in the last 5 years. Data included demographics (age, sex, laterality), region, time of presentation (year, season) and treatment received. RESULTS: The series included 3 males and 2 females. The mean age was 16 years. Three patients had presented in autumn of 2021, and two in autumn of 2019. Four patients presented within 2 weeks of the onset of symptoms. All patients had either exposure or contact with white moths. All patients presented with unilateral sudden painless reduction in vision and low Intraocular pressure. Four patients required vitrectomy. The final visual acuity of 3 patients was >6/36, and one patient was 6/60. CONCLUSIONS AND IMPORTANCE: The time of presentation and early treatment intervention are crucial in achieving good visual prognosis in SHAPU.
Subject(s)
Panuveitis , Uveitis , Male , Female , Humans , Adolescent , Seasons , Retrospective Studies , Bhutan/epidemiology , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/epidemiologyABSTRACT
PURPOSE: To evaluate the ocular features, course, and prognosis of uveitis in the ocular sarcoidosis (OS) subgroups without previously known systemic sarcoidosis. METHODS: Ninety-one eyes of 49 patients were included. Definite OS patients were classified as group 1 (n = 15), presumed OS patients as group 2 (n = 15), and probable OS patients as group 3 (n = 19). RESULTS: The most common presentation of OS was panuveitis in all groups. During the follow-up, bilateral ocular involvement was observed in 85.7% of the patients. The most common extraocular involvement was pulmonary involvement, which was detected in 61.2% of the patients at the time of diagnosis. CONCLUSION: Ophthalmologists should be aware that uveitis may be the first sign of systemic sarcoidosis. In all subgroups of OS, uveitis tends to be bilateral and the most common type is panuveitis. Therefore, the eye without obvious clinical findings should be carefully evaluated clinically and angiographically.
Subject(s)
Endophthalmitis , Panuveitis , Sarcoidosis , Uveitis , Humans , Tertiary Care Centers , Turkey/epidemiology , Sarcoidosis/diagnosis , Uveitis/diagnosis , Panuveitis/diagnosis , Panuveitis/drug therapy , Retrospective StudiesABSTRACT
PURPOSE: To report a case of programmed cell death receptor-1 (PD-1) inhibitor induced panuveitis. METHOD: Observational case report of a 13-year-old Chinese girl presented as panuveitis. The clinical course, imaging performance, laboratory examination, differential diagnosis, treatment and prognosis were described. RESULT: Patient presented with bilateral anterior granulomatous uveitis, vitritis, papillitis, and various creamy yellow nodular lesions in the mid-peripheral fundus. She had a history of biopsy proven alveolar soft tissue sarcoma on the chest wall and pulmonary metastasis, and a PD-1 inhibitor (sintilimab) was intravenously administered. Blood tests, magnetic resonance imaging of the cranium and the orbit, aqueous humor assay of inflammatory cytokines and microbial DNA were performed to distinguish infectious and non-infectious uveitis, choroidal metastases, and intravenous injection-related endophthalmitis. The oncologist evaluated that the sarcoma was stable and terminated sintilimab dosage. After sintilimab withdrawal, the blurred vision improved. Then, the patient received oral corticosteroids, resulted in resolution of the panuveitis. A diagnosis of PD-1 inhibitor induced panuveitis was made. CONCLUSION: For patients taking PD-1 inhibitors, the major diagnostic challenge is to identify whether the cause of the uveitis is due to the antitumor treatment or not. It is suggested to be screened by eye care specialist and timely referral to uveitis specialist with any suspicion of intraocular inflammation for these patients.
Subject(s)
Panuveitis , Sarcoma , Uveitis, Anterior , Uveitis , Female , Adolescent , Humans , Immune Checkpoint Inhibitors/adverse effects , Panuveitis/chemically induced , Panuveitis/diagnosis , Panuveitis/drug therapy , Inflammation , Sarcoma/diagnosis , Sarcoma/drug therapyABSTRACT
PURPOSE: To evaluate the effectiveness of tacrolimus in patients with noninfectious intermediate, posterior, or panuveitis needing a two-immunosuppressive-agent regimen. METHODS: Design: Retrospective cohort study. Setting: Two tertiary-care uveitis practices at academic medical centers. Patient population: Thirty-two patients with noninfectious intermediate, posterior, or panuveitides in whom single-agent immunosuppression was inadequate to effect successful corticosteroid sparing. Intervention: tacrolimus, added as the second immunosuppressive agent. Main outcome measure: successful corticosteroid sparing, defined as inactive uveitis at a dose of prednisone ≤7.5 mg/day. RESULTS: Active uveitis was present in 65.6% of patients at initiation of tacrolimus, and the median time to inactive uveitis was 1.5 months (95% confidence interval 1.2, 4.08). The median time to successful corticosteroid sparing was 3.9 months (95% confidence interval 1.41, 6.67), and by 6 months of follow-up successful corticosteroid sparing was achieved in 75% of patients. Tacrolimus was discontinued for side effects in five patients, three for tremor, and two for hyperglycemia. All side effects were reversible with tacrolimus discontinuation. CONCLUSION: Tacrolimus seems to have efficacy as a second immunosuppressive agent in two-immunosuppressive drug regimens, when a single agent does not permit successful corticosteroid sparing. Side effects were reversible with tacrolimus discontinuation.
Subject(s)
Panuveitis , Uveitis , Humans , Tacrolimus/therapeutic use , Retrospective Studies , Panuveitis/drug therapy , Uveitis/drug therapy , Immunosuppressive Agents/therapeutic use , Immunosuppression Therapy , Treatment OutcomeABSTRACT
PURPOSE: To report a case of bilateral panuveitis and occlusive vasculitis following COVID-19 vaccination. STUDY DESIGN: Case report. RESULTS: A 41-year-old otherwise healthy male presented with progressive vision loss and floaters starting 48 hours after a first dose of COVID-19 vaccine. Examination initially showed bilateral anterior uveitis, but this evolved into bilateral panuveitis with occlusive vasculitis despite topical corticosteroids over two weeks. The patient underwent extensive testing for other etiologies which were excluded. He was successfully treated with a gradual taper of topical and systemic corticosteroids leading to improvement of signs and symptoms. Follow-up is maintained for observation of avascular zones with possible neovascularization which could require laser as needed. CONCLUSIONS: The temporal association between vaccine and presentation makes this a plausible etiology. This remains a rare adverse event, but clinicians should be aware of this possibility to include it in their differential diagnosis when confronted with idiosyncratic ocular presentations.
Subject(s)
COVID-19 , Panuveitis , Vasculitis , Humans , Male , Adult , COVID-19 Vaccines/adverse effects , COVID-19/complications , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiology , Vasculitis/complications , Adrenal Cortex Hormones/therapeutic use , Vaccination/adverse effectsABSTRACT
AIM: We report a case of a young male who developed bilateral panuveitis after extensive tattooing. DESIGN: Case report. METHODS: A 22-year-old male with a history of inflamed tattoos presented with pain in both eyes and blurred vision in the left eye. Clinical examination showed ciliary congestion, flare, vitreous cells in both eyes, and posterior synechiae in the left eye. Optic nerve was swollen in both eyes. OCT scans demonstrated subretinal blood, associated with neurosensory macular detachment in the left eye. The skin tattoo biopsy showed a granulomatous inflammation without evidence of sarcoidosis. Long-term corticosteroid therapy allowed a regression of clinical signs and symptoms with full recovery. CONCLUSION: TAttoo Granulomas with Uveitis (TAGU) is a syndrome with numerous clinical presentations. In our case, optic nerve head oedema and subretinal hemorrhage at the posterior pole were the presentation signs. Ophthalmologists should always consider TAGU as a diagnosis in patients with a history of inflamed tattoos.
Subject(s)
Panuveitis , Sarcoidosis , Tattooing , Uveitis , Humans , Male , Young Adult , Adult , Tattooing/adverse effects , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiology , Skin/pathology , Sarcoidosis/diagnosis , Granuloma/complications , Uveitis/complicationsABSTRACT
PURPOSE: Comparison of sarcoid uveitis with other non-infectious uveitis treatment and visual outcomes. METHODS: Retrospective study of 287 eyes with sarcoid uveitis and 1517 eyes with other non-infectious uveitis (15,029 eye-years follow-up). RESULTS: Sarcoid uveitis patients presented at age 43.1 ± 0.8 years, and 66.2% were female. Panuveitis was the most frequent presentation (48.3%), and 90.1% were bilateral. Moderate visual loss (≤20/50) developed in 19 eyes (6.6%), and severe visual loss (≤20/200) in 13 eyes (4.5%). Sarcoid uveitis had better visual outcomes than other non-infectious uveitis (10-year BCVA anterior uveitis 0.06 vs 0.24 p = .002; posterior disease 0.17 vs 0.38 p = .001). Oral corticosteroid use was more common with sarcoid uveitis (anterior uveitis 45.9% vs 16.4% p < .0005; posterior disease 64.0% vs 61.7% p = .635), but second-line immunosuppression was required less frequently (p = .008). CONCLUSIONS: Compared to other non-infectious uveitis, sarcoid uveitis has better visual acuity outcomes and is less likely to require second-line immunosuppression.
Subject(s)
Panuveitis , Sarcoidosis , Uveitis, Anterior , Uveitis , Humans , Female , Adult , Male , Retrospective Studies , Uveitis/diagnosis , Uveitis/drug therapy , Uveitis/etiology , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiology , Sarcoidosis/complications , Sarcoidosis/diagnosis , Sarcoidosis/drug therapy , Vision DisordersABSTRACT
PURPOSE: Morvan syndrome (MoS) is a neurologic disorder belonging to a spectrum of autoimmune encephalitis, Contactin-associated protein-like 2 (Caspr2) antibody syndrome. We report a case of bilateral panuveitis associated with MoS. METHODS: Case report and review of the literature. RESULTS: A 57-year-old male with Morvan syndrome presented with painless vision loss and floaters. Initial visual acuities were 20/50 and 20/60. Hallmarks of this uveitis included persistently active vitritis, and nonhemorrhagic retinitis with nonperfusion and neovascularization. Uveitis consistently flared with attempted immunosuppressive tapers. Vision deteriorated to count fingers (2 ft) OU over 2.5 years despite corticosteroids, mycophenolate mofetil, intravenous immunoglobulin, adalimumab, and rituximab. Explanations for reduced final visual acuity included macular atrophy, disruption of retinal architecture, epiretinal membrane, vitritis, and cataract. CONCLUSIONS: This case constitutes the first report of uveitis associated with MoS and Caspr2 antibody syndrome, raising the question of autoimmunity targeting the retinal inner and/or outer plexiform layers.
Subject(s)
Encephalitis , Panuveitis , Uveitis , Male , Humans , Middle Aged , Panuveitis/complications , Panuveitis/diagnosis , Panuveitis/drug therapy , Uveitis/etiology , Adrenal Cortex Hormones , Retina , Encephalitis/complications , Syndrome , Vision DisordersABSTRACT
BACKGROUND/AIM: This study evaluated real-life adalimumab impact in patients with active non-infectious intermediate, posterior, or panuveitis (NIIPPU). METHODS: Adults with active NIIPPU received adalimumab in this prospective, observational study (06/2017-04/2020). Patients were evaluated at baseline (V0) and four follow-up visits over 12 months (V1-V4). PRIMARY ENDPOINT: proportion of patients achieving quiescence (anterior chamber (AC) cells grade and vitreous haze (VH) grade≤0.5+ in both eyes, no new active chorioretinal lesions) at any follow-up visit. Secondary endpoints: proportion of patients achieving quiescence at each visit; proportion of patients maintaining response; and proportion of patients with flares. Workability, visual function, healthcare resource utilisation, and safety were evaluated. RESULTS: Full analysis set included 149 patients. Quiescence at any follow-up visit was achieved by 129/141 (91%) patients. Quiescence at individual visits was achieved by 99/145 (68%), 110/142 (77%), 102/131 (78%), and 99/128 (77%) patients at V1-V4, respectively. Number of patients in corticosteroid-free quiescence increased from 51/147 (35%; V1) to 67/128 (52%; V4; p<0.05). Proportion of patients with maintained response increased from 89/141 (63%; V2) to 92/121 (76%; V4; p<0.05) and proportion of patients with flare decreased from 25/145 (17%; V1) to 13/128 (10%; V4; p=0.092). Workability and visual function improved throughout the study. Proportion of patients with medical visits for uveitis decreased from 132/149 (89%; V0) to 27/127 (21%; V4). No new safety signals were observed. CONCLUSION: These results demonstrated adalimumab effectiveness in improving quality of life while reducing economic burden of active NIIPPU.
Subject(s)
Panuveitis , Uveitis , Adult , Humans , Adalimumab/therapeutic use , Quality of Life , Prospective Studies , Panuveitis/drug therapy , Treatment OutcomeABSTRACT
PURPOSE: Clinical trials in uveitis have led to the expansion of therapeutic options for the management of non-infectious uveitis. The purpose of this systematic review is to investigate why some clinical trials have yielded successful results and regulatory approval of new therapies, and some have not. METHODS: A systematic literature search of the Pubmed/MEDLINE database and clinicaltrials.gov was performed from 2006 to 2021, according to the PRISMA guidelines. Phase III clinical trials of systemic and local therapies in adults with non-infectious intermediate, posterior, and panuveitis were included. RESULTS: A total of 79 clinical trials were collected from ClinicalTrials.gov and PubMed/MEDLINE database search. Based on the inclusion and exclusion criteria, 14 clinical trials were included. CONCLUSION: This review summarizes the study design, outcome measures, and results of recent phase III trials in non-infectious uveitis, in the interest of understanding limitations and rethinking new methods of defining endpoints in clinical trial design.
Subject(s)
Panuveitis , Uveitis , Adult , Humans , Uveitis/drug therapy , Panuveitis/drug therapy , Outcome Assessment, Health Care , Visual AcuityABSTRACT
PURPOSE: To report a case of severe, recurrent bilateral panuveitis secondary to primary progressive multiple sclerosis responsive to ocrelizumab infusions. OBSERVATION: We describe the clinical progression of a 40 year old female who presented with a 3-week history of insidious bilateral visual loss that was clinically consistent with panuveitis. A diagnosis of multiple sclerosis was established with serial magnetic resonance imaging (MRI) that coincided with focal neurological events separated by time. There was initially good response to high dose oral prednisolone; however, the patient would have recurrent uveitis each time the dose was weaned. Under guidance of neurology, we had initiated treatment with ocrelizumab with stability of ocular inflammation for the past 24 months. CONCLUSION: Six-monthly 600mg ocrelizumab infusions may be effective as a steroid sparing option for patients with severe, recurrent bilateral panuveitis secondary to primary progressive multiple sclerosis.
Subject(s)
Multiple Sclerosis, Chronic Progressive , Multiple Sclerosis , Panuveitis , Uveitis , Female , Humans , Adult , Multiple Sclerosis/drug therapy , Multiple Sclerosis, Chronic Progressive/complications , Multiple Sclerosis, Chronic Progressive/diagnosis , Multiple Sclerosis, Chronic Progressive/drug therapy , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiologyABSTRACT
Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) remains a serious pandemic. COVID-19 vaccination is urgent needed for limiting SARS-CoV-2 outbreaks by herd immunity. Simultaneously, post-marketing surveillance to assess vaccine safety is important, and collection of vaccine-related adverse events has been in progress. Vision-threatening ophthalmic adverse events of COVID-19 vaccines are rare but are a matter of concern. We report a 45-year-old Japanese male with positive for HLA-DR4/HLA-DRB1*0405, who developed bilateral panuveitis resembling Vogt-Koyanagi-Harada (VKH) disease after the second dose of Pfizer-BioNTech COVID-19 mRNA (BNT162b2) vaccine. Glucocorticosteroid (GC) therapy combined with cyclosporine A (CsA) readily improved the panuveitis. The immune profile at the time of onset was analyzed using CyTOF technology, which revealed activations of innate immunity mainly consisting of natural killer cells, and acquired immunity predominantly composed of B cells and CD8+ T cells. On the other hand, the immune profile in the remission phase was altered by GC therapy with CsA to a profile composed primarily of CD4+ cells, which was considerably similar to that of the healthy control before the vaccination. Our results indicate that BNT162b2 vaccine may trigger an accidental immune cross-reactivity to melanocyte epitopes in the choroid, resulting in the onset of panuveitis resembling VKH disease.
Subject(s)
COVID-19 , Panuveitis , Uveomeningoencephalitic Syndrome , BNT162 Vaccine , CD8-Positive T-Lymphocytes , COVID-19 Vaccines/adverse effects , Cyclosporine/therapeutic use , Epitopes , HLA-DR4 Antigen , Humans , Male , Middle Aged , Panuveitis/diagnosis , Panuveitis/drug therapy , Panuveitis/etiology , RNA, Messenger/therapeutic use , SARS-CoV-2 , Uveomeningoencephalitic Syndrome/diagnosis , Uveomeningoencephalitic Syndrome/drug therapy , Uveomeningoencephalitic Syndrome/etiologyABSTRACT
BACKGROUND: Non-infectious intermediate, posterior, and panuveitis (NIIPPU) represent a heterogenous collection of autoimmune and inflammatory disorders isolated to or concentrated in the posterior structures of the eye. Because NIIPPU is typically a chronic condition, people with NIIPPU frequently require treatment with steroid-sparing immunosuppressive therapy. Methotrexate, mycophenolate, cyclosporine, azathioprine, and tacrolimus are non-biologic, disease-modifying antirheumatic drugs (DMARDs) which have been used to treat people with NIIPPU. OBJECTIVES: To compare the effectiveness and safety of selected DMARDs (methotrexate, mycophenolate mofetil, tacrolimus, cyclosporine, and azathioprine) in the treatment of NIIPPU in adults. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register), MEDLINE, Embase, the Latin American and Caribbean Health Sciences database, ClinicalTrials.gov, and the World Health Organization International Clinical Trials Registry Platform, most recently on 16 April 2021. SELECTION CRITERIA: We included randomized controlled trials (RCTs) comparing selected DMARDs (methotrexate, mycophenolate, tacrolimus, cyclosporine, and azathioprine) with placebo, standard of care (topical steroids, with or without oral steroids), or with each other. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included 11 RCTs with a total of 601 participants in this review. DMARDs versus control Two studies compared an experimental DMARD (cyclosporine A or enteric-coated mycophenolate [EC-MPS]) plus oral steroid with steroid monotherapy. We did not pool these results into a meta-analysis because the dose of cyclosporine used was much higher than that used in current clinical practice. The evidence is very uncertain about whether EC-MPS plus low-dose oral steroid results in a higher proportion of participants achieving control of inflammation over steroid monotherapy (risk ratio [RR] 2.81, 95% confidence interval [CI] 1.10 to 7.17; 1 study, 41 participants; very low-certainty evidence). The change in best-corrected visual acuity (BCVA) was reported separately for right and left eyes. The evidence for improvement (lower logarithm of the minimum angle of resolution (logMAR) indicates better vision) between the groups is very uncertain (mean difference [MD] -0.03 and -0.10, 95% CI -0.96 to 0.90 and -0.27 to 0.07 for right and left, respectively; 1 study, 82 eyes; very low-certainty evidence). No data were available for the following outcomes: proportion of participants achieving a 2-line improvement in visual acuity, with confirmed macular edema, or achieving steroid-sparing control. The evidence for the proportion of participants requiring cessation of medication in the DMARD versus control group is very uncertain (RR 2.61, 95% CI 0.11 to 60.51; 1 study, 41 participants; very low-certainty evidence). Methotrexate versus mycophenolate We were able to combine two studies into a meta-analysis comparing methotrexate versus mycophenolate mofetil. Methotrexate probably results in a slight increase in the proportion of participants achieving control of inflammation, including steroid-sparing control, compared to mycophenolate at six months (RR 1.23, 95% CI 1.01 to 1.50; 2 studies, 261 participants; moderate-certainty evidence). Change in BCVA was reported per eye and the treatments likely result in little to no difference in change in vision (MD 0.01 logMAR higher [worse] for methotrexate versus mycophenolate; 2 studies, 490 eyes; moderate-certainty evidence). No data were available for the proportion of participants achieving a 2-line improvement in visual acuity. The evidence is very uncertain regarding the proportion of participants with confirmed macular edema between methotrexate versus mycophenolate (RR 0.49, 95% CI 0.19 to 1.30; 2 studies, 35 eyes; very low-certainty). Methotrexate versus mycophenolate may result in little to no difference in the proportion of participants requiring cessation of medication (RR 0.99, 95% CI 0.43 to 2.27; 2 studies, 296 participants; low-certainty evidence). Steroids with or without azathioprine versus cyclosporine A Four studies compared steroids with or without azathioprine (oral steroids, intravenous [IV] steroids, or azathioprine) to cyclosporine A. We excluded two studies from the meta-analysis because the participants were treated with 8 mg to 15 mg/kg/day of cyclosporine A, a significantly higher dose than is utilized today because of concerns for nephrotoxicity. The remaining two studies were conducted in all Vogt-Koyanagi-Harada disease (VKH) populations and compared cyclosporine A to azathioprine or IV pulse-dose steroids. The evidence is very uncertain for whether the steroids with or without azathioprine or cyclosporine A influenced the proportion of participants achieving control of inflammation (RR 0.84, 95% CI 0.70 to 1.02; 2 studies, 112 participants; very low-certainty evidence), achieving steroid-sparing control (RR 0.64, 95% CI 0.33 to 1.25; 1 study, 21 participants; very low-certainty evidence), or requiring cessation of medication (RR 0.85, 95% 0.21 to 3.45; 2 studies, 91 participants; very low-certainty evidence). The evidence is uncertain for improvement in BCVA (MD 0.04 logMAR lower [better] with the steroids with or without azathioprine versus cyclosporine A; 2 studies, 91 eyes; very low-certainty evidence). There were no data available (with current cyclosporine A dosing) for the proportion of participants achieving a 2-line improvement in visual acuity or with confirmed macular edema. Studies not included in synthesis We were unable to include three studies in any of the comparisons (in addition to the aforementioned studies excluded based on historic doses of cyclosporine A). One was a dose-response study comparing cyclosporine A to cyclosporine G, a formulation which was never licensed and is not clinically available. We excluded another study from meta-analysis because it compared cyclosporine A and tacrolimus, considered to be of the same class (calcineurin inhibitors). We were unable to combine the third study, which examined tacrolimus monotherapy versus tacrolimus plus oral steroid, with any group. AUTHORS' CONCLUSIONS: There is a paucity of data regarding which DMARD is most effective or safe in NIIPPU. Studies in general were small, heterogenous in terms of their design and outcome measures, and often did not compare different classes of DMARD with each other. Methotrexate is probably slightly more efficacious than mycophenolate in achieving control of inflammation, including steroid-sparing control (moderate-certainty evidence), although there was insufficient evidence to prefer one medication over the other in the VKH subgroup (very low-certainty evidence). Methotrexate may result in little to no difference in safety outcomes compared to mycophenolate.
Subject(s)
Antirheumatic Agents , Macular Edema , Panuveitis , Adult , Humans , Macular Edema/etiology , Cyclosporine/therapeutic use , Mycophenolic Acid/therapeutic use , Tacrolimus/therapeutic use , Azathioprine/therapeutic use , Methotrexate/therapeutic use , Steroids/therapeutic use , Immunosuppressive Agents/therapeutic use , Panuveitis/complications , Panuveitis/drug therapy , Inflammation , Antirheumatic Agents/therapeutic useABSTRACT
To describe the clinical features, longitudinal pattern, and incidence rate of improvement of visual acuity (VA) following antibiotic therapy in patients with syphilitic uveitis. A total of 36 patients were included in this retrospective study from 2009 to 2020. The longitudinal patterns of mean VA values during follow-up were analyzed using a linear mixed model. Most patients were men with HIV coinfection (81%) and presented with panuveitis (49%). The mean VA at baseline improved from 0.97 to 0.39 logMAR at 6 months and remained stable thereafter. The cumulative incidence of VA ≥ 20/25 achieved by 2 years was 70%. Receiving antibiotic therapy within four weeks of the onset of ocular symptoms (adjusted hazard ratio [aHR] 3.4, P = 0.012), absence of HIV coinfection (aHR 8.2, P < 0.001), absence of neurosyphilis (aHR 6.5, P = 0.037), better presenting VA (aHR 5.0, P = 0.003), and intermediate uveitis as opposed to panuveitis (aHR 11.5, P = 0.013) were predictive of achieving VA ≥ 20/25. Men with HIV coinfection represented the majority of our patients. Visual outcomes, in response to antibiotics, were favorable. Delayed treatment, poor presenting VA, presence of HIV coinfection, and concomitant neurosyphilis decreased the likelihood of VA restoration.