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Journal of Ophthalmic Inflammation and... Mar 2024Ocular inflammatory diseases, including scleritis and uveitis, have been widely treated with immunomodulatory therapies (IMTs) as a steroid-sparing approach. Such...
BACKGROUND
Ocular inflammatory diseases, including scleritis and uveitis, have been widely treated with immunomodulatory therapies (IMTs) as a steroid-sparing approach. Such strategy includes conventional therapies (antimetabolites, alkylating agents, and calcineurin inhibitors) as well as biologic agents like adalimumab, infliximab, rituximab, and tocilizumab. Cyclophosphamide (CP) is an alkylating agent and mainly inhibits the functioning of both T and B cells. Though known to have potential adverse events, including bone marrow suppression, hemorrhagic cystitis, and sterility, CP has been shown to be efficacious, especially in recalcitrant cases and when used intravenous (IV) for a limited period.
MAIN FINDINGS
We conducted a retrospective case-series to assess the safety and efficacy of CP therapy for patients with severe ocular inflammatory diseases who failed other IMTs. Medical records of 1295 patients who presented to the Uveitis Clinic at the Byers Eye Institute at Stanford between 2017 and 2022 were reviewed. Seven patients (10 eyes) who received CP therapy for ocular inflammatory diseases with at least one year of follow-up were included. The mean age of the patients (4 males, 3 females) was 61.6 ± 14.9 (43.0-89.0) years. Clinical diagnoses included necrotizing scleritis (5 eyes), peripheral ulcerative keratitis (2 eyes), orbital pseudotumor (1 eye), HLA-B27 associated panuveitis and retinal vasculitis (2 eyes). Ocular disease was idiopathic in 3 patients, and was associated with rheumatoid arthritis, IgG-4 sclerosing disease, dermatomyositis, and ankylosing spondylitis in 1 patient each. All the patients had history of previous IMT use including methotrexate (5), mycophenolate mofetil (3), azathioprine (1), tacrolimus (1), adalimumab (2), infliximab (4), and rituximab (1). The mean follow-up time was 34.4 ± 11.0 (13-45) months, and mean duration of CP therapy was 11.9 ± 8.8 (5-28) months. Remission was achieved in 5 patients (71.4%). Four patients (57.1%) experienced transient leukopenia (white blood cell count < 4000/mL).
SHORT CONCLUSION
CP therapy can be considered a potentially effective and relatively safe therapeutic option for patients with severe ocular inflammatory diseases who failed other IMTs including biologics (TNFa and CD20 inhibitors).
PubMed: 38466527
DOI: 10.1186/s12348-023-00372-z -
Journal of Surgical Case Reports Mar 2024Dabrafenib and trametinib, approved for the treatment of BRAF-mutant metastatic melanoma, are associated with a spectrum ophthalmic toxicity including pan-uveitis and...
Dabrafenib and trametinib, approved for the treatment of BRAF-mutant metastatic melanoma, are associated with a spectrum ophthalmic toxicity including pan-uveitis and serous retinopathy. Vogt-Koyanagi-Harada (VKH) is a systemic inflammatory disorder characterized by bilateral granulomatous pan-uveitis, exudative retinal detachments, and often associated with extraocular manifestations such as tinnitus, vitiligo, headaches, or encephalopathy. We present a 49-year-old woman with stage IV metastatic cutaneous melanoma developed bilateral acute pan-uveitis with multifocal serous retinal detachments, 4 months after starting combined dabrafenib and trametinib therapy. Clinical assessment, together with fluorescein angiography, optical coherence tomography, and serology led to the diagnosis of a (VKH)-like uveitis. Prompt systemic corticosteroids and modification of the dosing schedule of the suspected offending agents resulted in the resolution of intraocular inflammation and serous retinal detachments. This case underscores the importance of the prompt recognition of the association between VKH-like uveitis and BRAF/MEK inhibitors, enabling early intervention without compromising metastatic melanoma treatment.
PubMed: 38455982
DOI: 10.1093/jscr/rjae104 -
Indian Journal of Ophthalmology Jul 2024To evaluate the spectrum of uveitis occurring after 60 years of age in elderly patients who presented to a tertiary care eye center in India.
PURPOSE
To evaluate the spectrum of uveitis occurring after 60 years of age in elderly patients who presented to a tertiary care eye center in India.
METHODS
Retrospective study of patients who visited a tertiary eye care institute between January 2010 and July 2020.
RESULT
Eighty-seven patients developed uveitis after 60 years, with only 44.8% having sufficient follow-up documentation and were included in the final analysis. The median age of these patients was 64 (IQR: 62-70) years, and 69% of them were male. Among the identifiable causes of uveitis, infectious uveitis (36%) was the most common and noninfectious uveitis was noted in 23% of patients. The most common subtype of uveitis was anterior uveitis (52%), followed by intermediate uveitis (32%), panuveitis (11%), and posterior uveitis (7%). Tuberculosis (28%) was the most common cause in our cohort, followed by HLA B27 (10%), sarcoid (8%), and Vogt-Koyanagi-Harada disease (5%). In 41% of patients, a definitive diagnosis of uveitis could not be achieved, and the anterior uveitis group had the highest number of undifferentiated uveitis cases. There were no undifferentiated cases of uveitis in the posterior and panuveitis category. The median follow-up period of these patients was 52 (15-91) months and 66% of eyes had recurrence. A statistically significant improvement in vision was seen in anterior uveitis and panuveitis groups, whereas the median visual acuity of the intermediate uveitis group remained stable throughout the follow-up period.
CONCLUSION
Uveitis in the elderly can have a higher recurrence rate; however, the lack of follow-up in these groups of patients is a major challenge.
Topics: Humans; Male; India; Retrospective Studies; Female; Aged; Middle Aged; Uveitis; Follow-Up Studies; Visual Acuity; Incidence
PubMed: 38454869
DOI: 10.4103/IJO.IJO_2788_23 -
Indian Journal of Ophthalmology Jul 2024Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults,... (Review)
Review
Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults, showing a female predominance. This condition, distinct from genetic disorders like retinitis pigmentosa, lacks a hereditary basis and exhibits unique fundus changes and imaging features indicative of outer retinal dysfunction. Recent advancements have broadened our understanding, identifying variants like Acute Annular Outer Retinopathy (AAOR) and Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR), each with specific clinical presentations and imaging characteristics. The diagnosis of AZOOR and its variants primarily relies on excluding other conditions through comprehensive evaluation, including imaging and serological testing. Treatment approaches, including the use of corticosteroids and immunosuppressives, remain debated, with some evidence suggesting benefits in the early stages. The prognosis of AZOOR varies, with most patients experiencing stabilization, although complications like choroidal neovascularization may occur, requiring targeted therapy. This manuscript elucidates the complexity of AZOOR, emphasizing the necessity of high clinical suspicion and the role of advanced imaging in diagnosis and management.
Topics: Humans; White Dot Syndromes; Scotoma; Tomography, Optical Coherence; Fluorescein Angiography; Fundus Oculi; Visual Acuity; Visual Fields; Electroretinography
PubMed: 38454854
DOI: 10.4103/IJO.IJO_3228_23 -
Indian Journal of Ophthalmology Jul 2024To compare various ocular thermography parameters in posterior scleritis (PS), choroiditis (choroidal granuloma [CG], Vogt-Koyanagi-Harada [VKH] syndrome), central... (Comparative Study)
Comparative Study Observational Study
PURPOSE
To compare various ocular thermography parameters in posterior scleritis (PS), choroiditis (choroidal granuloma [CG], Vogt-Koyanagi-Harada [VKH] syndrome), central serous chorioretinopathy (CSCR), and healthy controls.
METHODS
This retrospective, observational, comparative study evaluated cases undergoing ocular thermography between April 2017 and October 2023. The study groups included cases of PS, CG, and VKH, while the control group comprised CSCR cases and healthy individuals. Various thermography parameters were assessed, which were as follows: Ocular surface temperature (OST), central corneal temperature (CCT), average scleral temperature (ST), nasal scleral temperature (nST), temperature difference between both the eyes (∆t), and difference between scleral and corneal temperatures (ST - CCT, nST - CCT).
RESULTS
It was found that ∆t was significantly higher in the PS group compared to the CG ( P = 0.005), CSCR ( P = 0.0001), and control groups (dilated control: P =0.006, undilated control: P = 0.0001). ST - CCT and nST - CCT were significantly higher in the undilated control and CSCR groups and significantly lower in the PS group. ST - CCT and nST - CCT showed less difference in the affected eyes compared to contralateral healthy eyes of PS and CG cases. OST, CCT, ST, and nST displayed statistically insignificant differences across all groups.
CONCLUSION
It is advisable to focus on temperature differences between the affected and healthy eyes, or the difference between the central corneal and scleral temperature of the affected eye, utilizing parameters such as ∆t, ST - CCT, and nST - CCT. These composite parameters offer a more effective approach than individual measurements like OST, CCT, ST, and nST. Thermography can serve as a screening tool to suspect and differentiate PS.
Topics: Humans; Scleritis; Retrospective Studies; Thermography; Female; Male; Adult; Middle Aged; Sclera; Choroiditis; Body Temperature
PubMed: 38454839
DOI: 10.4103/IJO.IJO_2830_23 -
BMC Ophthalmology Mar 2024To report a case of unusual presentation of retinochoroiditis caused by Rickettsia typhi in a patient without prior uveitis.
BACKGROUND
To report a case of unusual presentation of retinochoroiditis caused by Rickettsia typhi in a patient without prior uveitis.
CASE PRESENTATION
In this case, we describe a 24-year-old male soldier with no previous eye disease, who was referred to our ophthalmology department due to bilateral retinochoroiditis and vitritis. The patient initially presented with a paracentral scotoma in his right eye persisting for 7 days and scattered dark spots in his left eye for 2 days in June 2023. Preceding these ocular symptoms, he experienced a two-week episode of fever, headaches, night sweats, and rapid weight loss of 10 kg. A transient rash covered his body briefly. His mother had a history of recurrent eye inflammation. Physical examination revealed bilateral keratic precipitates on the lower corneal periphery, 1 + anterior vitreous cells, small retinal lesions and mild optic discs elevation. Fluorescein angiography indicated mild discs hyperfluorescence, and the clinically visible round punctate lesions on OCT showed inner retinal hyper-reflective lesion with a depth till outer plexiform layer possibly suggestive of a retinitis lesion. Laboratory tests were normal except thrombocytosis, elevated ESR, liver enzymes and ACE levels, with positive Rickettsia typhi serology tests. Rheumatology and infectious disease consultations ruled out autoimmune diseases, confirming Rickettsia typhi infection. Treatment included systemic doxycycline and prednisone, with improvement of visual acuity, ocular symptoms, OCT abnormalities and resolution of inflammation. Prednisone was discontinued, and after two months, additional improvement was seen clinically, with preserved retinal structures on OCT.
CONCLUSION
This study explores retinochoroiditis as a rare ocular presentation of Rickettsia typhi, an unusual infection in the Middle East. Previously reported ocular manifestations include conjunctivitis, vitritis, post infectious optic neuropathy and a few cases of uveitis. Ocular symptoms followed systemic illness, highlighting the need for awareness among clinicians. Diagnosis relies on seroconversion, with fluorescein angiography and OCT aiding in assessment. Empiric doxycycline and systemic corticosteroid therapy is recommended. Ocular symptoms resolved in two months. Awareness of these ocular manifestations is essential for timely diagnosis and management. Further research is needed to fully understand this aspect of murine typhus.
Topics: Humans; Male; Young Adult; Chorioretinitis; Doxycycline; Inflammation; Prednisone; Typhus, Endemic Flea-Borne
PubMed: 38454387
DOI: 10.1186/s12886-024-03329-5 -
BMC Ophthalmology Mar 2024Frosted branch angiitis is a retinal vascular condition that is associated with a viral infection or autoimmune disorders like Crohn's disease, systemic lupus...
BACKGROUND
Frosted branch angiitis is a retinal vascular condition that is associated with a viral infection or autoimmune disorders like Crohn's disease, systemic lupus erythematosus, and Behcet's disease. Frosted branch angiitis presents with vascular inflammation, retinal edema, and severe retinal vascular sheathing. We present a case of systemic juvenile idiopathic arthritis, an autoinflammatory disease, presenting with frosted branch angiitis.
REPORT OF CASE
A 14-year-old female with systemic juvenile idiopathic arthritis and a history of bilateral anterior uveitis developed acute unilateral vision loss and was found to have frosted branch angiitis complicated by branch retinal vein occlusion. She underwent an extensive serology workup and aqueous viral PCR to rule out other possible autoimmune and viral etiologies for forested branch angiitis. She received systemic and intravitreal antiviral treatment due to positive CMV IgM initially. However, the clinical picture improved following the use of a higher dose of oral steroids and the switch of the immunosuppressive agent to a TNF-a inhibitor.
CONCLUSION
To our knowledge, this would be the first case in the literature demonstrating a systemic juvenile idiopathic arthritis patient presenting with frosted branch angiitis. Infectious causes still must be ruled out, especially CMV, as it is the most common cause of secondary frosted branch angiitis.
Topics: Female; Humans; Adolescent; Arthritis, Juvenile; Vasculitis; Behcet Syndrome; Immunosuppressive Agents; Retinal Diseases; Cytomegalovirus Infections
PubMed: 38443873
DOI: 10.1186/s12886-024-03373-1 -
Middle East African Journal of... 2023The purpose was to study the anatomical and functional outcome following single low-dose suprachoroidal triamcinolone acetonide (LD-SCTA) (2 mg) injection in...
PURPOSE
The purpose was to study the anatomical and functional outcome following single low-dose suprachoroidal triamcinolone acetonide (LD-SCTA) (2 mg) injection in noninfectious posterior uveitis.
METHODS
Eleven patients with macular edema (ME) more than 280 μ secondary to noninfectious uveitis were included in the study. A single LD-SCTA (0.5 ml) injection was performed in the study eye with the help of a novel suprachoroidal microneedle (Pricon, Iscon Surgicals, Jodhpur, Rajasthan, India). The study parameters were noted at 4 and 12 weeks post LD-SCTA injection.
RESULTS
Ten of 11 patients had a significant decrease in central macular thickness (CMT). The mean CMT measurement at baseline was 513.6 ± 191.73 μm for the 10 patients who responded to the treatment, which reduced significantly to 265.1 ± 34.72 μm ( < 0.003) and 260.6 ± 34.72 μm ( < 0.002) at 4 and 12 weeks, respectively. The mean best-corrected visual acuity (BCVA) at baseline was 0.84 ± 0.41 logMAR unit which improved to 0.52 ± 0.33 ( < 0.001) and 0.25 ± 0.22 ( < 0.000) at weeks 4 and 12, respectively. The mean intraocular pressure at baseline recorded was 16.36 ± 2.97 mmHg, 19.45 ± 4.80 mmHg ( = 0.06) at 4 weeks, and 17.27 ± 2.53 mmHg ( = 0.35) at 12 weeks. One eye which did not respond to LD-SCTA was a case of recurrent Vogt-Koyanagi-Harada disease.
CONCLUSION
Single LD-SCTA injection is efficacious in reducing CMT in ME, improving BCVA, and controlling the inflammation in noninfectious posterior uveitis. LD-SCTA can be used as a first-line therapy in noninfectious uveitis over other routes of steroid administration with a favorable outcome and safety profile.
Topics: Humans; Triamcinolone Acetonide; Macular Edema; India; Uveitis, Posterior; Uveomeningoencephalitic Syndrome
PubMed: 38435103
DOI: 10.4103/meajo.meajo_78_21 -
Ceska a Slovenska Oftalmologie :... 2024We present the clinical spectrum, the initial clinical presentation with management trends in treating 14 Vogt-Koyanagi-Harada (VKH) disease cases in a tertiary center...
AIMS
We present the clinical spectrum, the initial clinical presentation with management trends in treating 14 Vogt-Koyanagi-Harada (VKH) disease cases in a tertiary center in the Northern part of Malaysia.
CASE SERIES
There were 14 cases of Vogt-Koyanagi-Harada (VKH) disease retrospectively reviewed over five years (from 2015 to 2020). The mean age at presentation was 37.7 years (range 21-64 years), with female predominance (85.7%). All cases presented with acute uveitic stage and bilateral eye involvement. Of them, 11 (78.6%) were probable VKH, and three (21.4%) were incomplete VKH. All patients attended with acute panuveitis at first presentation. The main posterior segment involvement was disc edema in 57.1% (16 out of 28 eyes) and exudative retinal detachment (ERD) in 35.7% (10 out of 28 eyes). Most of them presented with blindness (3/60 and worse) and moderate visual impair- ment (6/18-6/60); 35.71% each, followed by mild visual impairment (6/12-6/18), and severe visual impairment (6/60-3/60); 7.1% each. Ten patients (71.4%) required combination second-line immunomodulatory treatment during subsequent visits, and only four patients (28.6%) responded well to corticosteroid therapy. Most of the cases achieved no visual impairment (64.3%), followed by mild visual impairment (21.4%) and moderate visual impairment (14.3%), and none were severe or blind at the end of follow-up.
CONCLUSION
VKH is a potentially blinding illness if there is inadequate control of the disease in the acute stage. Most of our patients achieved good visual outcomes with early immunomodulatory treatment and systemic corticosteroids.
Topics: Humans; Female; Young Adult; Adult; Middle Aged; Male; Uveomeningoencephalitic Syndrome; Retrospective Studies; Malaysia; Glucocorticoids; Vision Disorders; Blindness
PubMed: 38413229
DOI: 10.31348/2024/1 -
Frontiers in Immunology 2024Recent studies have demonstrated an increased incidence of ischemic stroke among patients with certain autoimmune inflammatory rheumatic diseases (AIIRDs). However, the...
BACKGROUND
Recent studies have demonstrated an increased incidence of ischemic stroke among patients with certain autoimmune inflammatory rheumatic diseases (AIIRDs). However, the associations between young stroke and AIIRDs have not been fully investigated. This study aimed to evaluate the risk of ischemic stroke among young patients with AIIRDs.
METHODS
The National Health Insurance Research Database in Taiwan was utilized to establish cohorts of patients with AIIRDs diagnosed between 2004 and 2015, who were compared with 1,000,000 control participants. Cox proportional hazards regression models were used to calculate the hazard ratio of ischemic stroke and young ischemic stroke for individual AIIRDs after adjustment for relative risk factors.
RESULTS
During the study period, a total of 64,120 patients with AIIRDss and 1,000,000 control patients were identified. The overall mean follow-up time was 5.33 years. There were 223 (0.8%) and 1,923 (0.3%) young ischemic stroke-related hospitalizations among patients with AIIRDs and controls, respectively. The incidence rate of young ischemic stroke was 0.08 in patients with rheumatoid arthritis, 0.08 in patients with Sjögren's syndrome, 0.26 in patients with systemic lupus erythematosus, 0.17 in patients with idiopathic inflammatory myositis, 0.24 in patients with systemic sclerosis, 0.05 in patients with Behçet's disease, and 0.44 in patients with systemic vasculitis, versus 0.05 per 100 person-years in the general population. The adjusted hazard ratios for young ischemic stroke were 1.07 (95% CI 0.70-1.43) for rheumatoid arthritis, 1.39 (95% CI 0.94-2.06) for Sjögren's syndrome, 5.79 (95% CI 4.68-7.17) for systemic lupus erythematosus, 2.07 for idiopathic inflammatory myositis (95% CI 0.98-4.38), 2.79 for systemic sclerosis (95% CI 1.38-5.63), 0.82 for Behçet's disease (95% CI 0.26-2.55), and 4.15 (95% CI 1.96-8.82) for systemic vasculitis.
CONCLUSIONS
Patients younger than 50 years with systemic lupus erythematosus, systemic sclerosis, or systemic vasculitis have a significantly elevated risk of developing ischemic stroke. Further research is needed to elucidate the pathogenesis of accelerated atherosclerosis in these AIIRDs.
Topics: Humans; Sjogren's Syndrome; Ischemic Stroke; Cohort Studies; Behcet Syndrome; Taiwan; Arthritis, Rheumatoid; Lupus Erythematosus, Systemic; Scleroderma, Systemic; Rheumatic Fever; Myositis; Systemic Vasculitis
PubMed: 38404587
DOI: 10.3389/fimmu.2024.1272557