Retinal vasculitis, characterized by inflammation of retinal blood vessels, presents a significant challenge in diagnosis and management. Retinal vasculitis is a rare but potentially blinding condition that affects the retina and light-sensitive tissue at the back of the eye [1-4]. Understanding the etiopathogenesis, clinical course, and visual outcome of retinal vasculitis is crucial for the effective management and treatment of this condition, which can lead to vision loss if left untreated [1,3,5,6,7]. Previous studies have reported the clinical features and treatment options for retinal vasculitis, but there is limited research on the underlying causes and long-term outcomes of the condition [4,5,8,9]. There is a lack of comprehensive research on the etiopathogenesis of retinal vasculitis, and there is a need to investigate the factors that contribute to the development and progression of this condition, particularly in this region. This study aimed to address the knowledge gap in the field of retinal vasculitis by investigating the etiopathogenesis, clinical course, and visual outcomes of the condition in a tertiary care hospital setting. The present study aimed to determine the underlying causes and risk factors for retinal vasculitis and how they impact the clinical course and visual outcome of the condition. This study aimed to investigate the etiopathogenesis, clinical course, and visual outcome of retinal vasculitis in a tertiary care hospital setting. We hypothesized that retinal vasculitis is associated with certain underlying causes and risk factors, which may affect the clinical course and visual outcome of the condition.

After obtaining permission from the Institutional Ethics Committee, the present study was initiated. The present study is a prospective study and 100 cases of retinal vasculitis who attended the vitro-retinal clinic of Regional Institute of Ophthalmology, SCBMCH, Cuttack between October 2019 to September 2021 for a period of 2 years were taken up for the study. Inclusion criteria were age > 18 years, all cases of retinal vasculitis presenting with at least one of the following features: sheathing, perivascular inflammation, superficial and deep hemorrhages, and staining of vessel wall on FFA. Patients aged < 18 years with pre-existing ocular diseases such as diabetic retinopathy, vein occlusion, arterial occlusion, and glaucoma were excluded.

Patients presenting to the Retina Clinic were registered, evaluated, and followed up during the study period. A detailed history of the patient, visual acuity assessment, intraocular pressure measurement, slit lamp examination, and fundus examination were obtained. Fundus Fluorescein Angiography and Optical Coherence Tomography were performed in patients without a clear media. All patients were subjected to routine investigations, including complete hemogram, Mantoux test, random blood sugar, VDRL, and ELISA for HIV. All patients were referred to the respective departments attached to the SCBMCH Cuttack. Patients were managed according to their presentation with either conservative treatment or surgical intervention. Patients with peripheral vasculitis and good visual acuity with no evidence of retinal nonperfusion were advised to undergo periodic observation. Patients with peripheral vasculitis and good visual acuity with less than 5 DD retinal non-perfusion and systemic steroids (T. Prednisolone 1 mg/kg/day) were started which was tapered gradually. Immunosuppressants (azathioprine 2 mg/kg/day) were planned for resistant cases (not respond to steroid treatment for a period of 8 weeks). Patients with CME secondary to vasculitis received anti-VEGF injections. Patients with peripheral vasculitis and retinal non-perfusion of > 5 DD laser photocoagulation were advised. For patients with vitreous hemorrhage, observation and regular follow-up were advised. For patients with non-clearing vitreous hemorrhage and tractional retinal detachment, vitrectomy with endo-photocoagulation is advised. Screening procedures were performed, which included a detailed history of the present illness, visual acuity using Snellen’s chart, slit lamp biomicroscopy of the anterior segment, intraocular pressure using Goldmann Applanation tonometer, direct and indirect ophthalmoscopy, fundus fluorescein angiography, optical coherence tomography, and B scan. Blood tests were performed. All patients were planned for follow-up at 1^st, 2^nd, 4^th, 3^rd, 6^th, 9^th, and 12^th month and the outcomes at the end of 3 months were analyzed.

In our study, we observed that the incidence of retinal vasculitis was higher in males (80%) than that in females (20%).  Of the 62 patients with primary vasculitis, 60 were male and only 2 were female. Of the 8 patients with retinal vasculitis secondary to ocular disease, 6 were male and 2 were female. Of the 30 patients with retinal vasculitis secondary to systemic disease, 15 were males and 15 were females. We also observed cases due to primary retinal vasculitis were maximum (62%), followed by those due to systemic diseases (30%) and due to ocular disease (8%). We observed laterality predominantly in both eyes (54%), followed by the right eye (30%) and left eye (16%).

Acute onset was observed in 31% of the cases and chronic onset in 31% of the cases, that is, primary retinal vasculitis had an equal proportion of onset. In retinal vasculitis secondary to systemic disease, the mode of presentation was acute onset-15%, chronic onset-15%, retinal vasculitis due to ocular disease (2%), and asymptomatic (6 patients) (Table 1).

Table 1: Mode of presentation.

Tables 2 and 3 list the presenting complaints and clinical signs in the study population. Most of the patients presented with complaints of defective vision, in 40% of the cases associated with floaters and a few cases with defective vision plus redness or floaters with photophobia. In Table 3, we observed that patients with primary vasculitis mostly presented with perivascular sheathing and hemorrhages, those secondary to systemic diseases also presented maximally with perivascular sheathing and hemorrhages, and those secondary to ocular disease had sheathing as the predominant finding. Of the 62 cases of primary vasculitis, 12 patients had vitreous hemorrhage at the time of presentation, 18 had associated neovascularization, 14 had tractional bands, and 4 had tractional retinal detachment. Among the 30 cases secondary to systemic diseases, two patients had vitreous hemorrhages, six patients had neovascularization, and six patients had tractional bands. Eight cases were secondary to ocular disease.

Table 2. Presenting complaints in the study population.

Table 3: Clinical signs of the study population.

Table 4: Treatment Modality

Table 4 shows the treatment modalities used for the study population. Among patients with primary vasculitis, those who presented with vitreous hemorrhage, tractional retinal detachment, tractional bands, and minimal sheathing (24 patients) with no capillary non-perfusion area observation were advised. Patients with extensive sheathing and hemorrhages with capillary non-perfusion of less than 5 DD (22 patients) started treatment.

Table 5: BCVA before treatment and after treatment in the study population.

In our study population (table 5), in patients with primary vasculitis best corrected visual acuity at presentation was predominantly less than 6/60 in 56 of the cases. In patients with vasculitis secondary to ocular disease, the best-corrected visual acuity was equally present in the range of 6/24–6/60 and less than 6/60. In 34 patients with vasculitis secondary to systemic disease, the best-corrected visual acuity was less than 6/60. After treatment (Table 5), in patients with primary vasculitis, visual improvement was noted after 3 months of treatment, with 10 cases of eyes in the range of 6/6 – 6/18 as compared to the eyes of 6 patients before treatment. In the range of 6/24–6/60 post-treatment, there were 51.06% (48 eyes) as compared to 36.73% pre-treatment. The percentage of patients in the < 6/60 range reduced to 38.30% from the initial 57.15%. in vasculitis secondary to ocular disease, dramatic improvement was seen with 100% of patients improving in the range of 6/6 – 6/18 as compared to 0% pre-treatment. In vasculitis secondary to systemic disease, patients in the range of 6/6 – 6/18 initially increased to 18.18% compared to nil. The number of patients in the range of 6/24 – 6/60 increased to 50% from the initial 29.17%. The proportion of patients with visual acuity less than 6/60 initially reduced to 31.82% compared to 70.83 %. 4. Patients lost to follow-up.

The BCVA in logmar value changed from a mean of 1.4976 with a standard deviation of 0.8278 to 0.98961 with a standard deviation of 0.6495. A p-value < 0.001 signifies improvement in vision after 3 months of treatment and close observation.

One patient developed weight gain following steroid administration, 2 patients complained of gastritis following oral steroid administration, and 4 patients were lost to follow-up.

Retinal vasculitis, which features the inflammation of retinal blood vessels, presents a significant challenge in terms of diagnosis and management. This rare but potentially blinding condition affects not only the retina but also the light-sensitive tissues situated at the back of the eye. It is essential to comprehend the etiopathogenesis, clinical course, and visual outcomes of retinal vasculitis to manage and treat the condition effectively, as failure to do so can result in vision loss. This study aimed to investigate the etiopathogenesis, clinical course, and visual outcomes of retinal vasculitis in a tertiary care hospital setting. We hypothesized that retinal vasculitis is associated with certain underlying causes and risk factors that may influence the clinical course and visual outcome of the condition.

In this study, we analyzed the medical records of 100 patients who had been diagnosed with retinal vasculitis. Among these, 62 cases were classified as primary vasculitis, 30 as systemic diseases, and eight as ocular diseases.

At our clinic, we observed that the highest incidence of retinal vasculitis occurred in patients age group–18-28 years, followed by those age group–29-38 years. The mean age of the patients in our study was 30.02 ± 12 years. A study by [3] reported a mean age of 33 ± 11 years, while [7,8] reported a mean age of 28 years for men and 30 years for women. In our study, the incidence of retinal vasculitis was significantly higher in males (80%) than that in females (20%). Among the 62 patients with primary vasculitis, 60 were male and only 2 were female. Among the 8 patients with retinal vasculitis secondary to ocular disease, 6 were male and 2 were female. Among the 30 patients with retinal vasculitis secondary to systemic disease, 15 were male and 15 were female. The male predilection of this study was supported by another study [9] in which 93% of the participants were male. In our study, vasculitis secondary to systemic disease had an almost equal incidence in males and females.

We observed laterality predominantly in both eyes (54%), followed by the right eye (30%), and left eye (16%). Studies [10,11] have reported that 90% of the patients in their study were affected on both sides.

Most patients were presented with complaints of defective vision, in 40% of the cases associated with floaters and a few cases with defective vision plus redness or floaters with photophobia. Biswal et al., [3] observed defective vision as a commonest symptom in their study, in addition to floaters compared to our study.

We observed that patients with primary vasculitis mostly presented with perivascular sheathing and hemorrhages, those secondary to systemic diseases also presented maximally with perivascular sheathing and hemorrhages, and those secondary to ocular disease had sheathing as the predominant finding. Of the 62 cases of primary vasculitis, 12 had vitreous hemorrhage at the time of presentation, 18 had associated neovascularization, 14 had tractional bands, and 4 had tractional retinal detachment. Among the 30 cases secondary to systemic diseases, two patients had vitreous hemorrhages, six patients had neovascularization, and six patients had tractional bands. Biswal et al. [3] have reported vascular sheathing as the commonest sign in their study. In consultation with a previous study [3], another study [11] also observed that vascular sheathing involved veins more than arterial involvement. On the other hand, studies [12,13] observed vitreous hemorrhage as the most common finding in their study. 

Biswa et al. [3] reported that corticosteroids were the main treatment modality, which is like our study. After a follow-up period of three months, the patients’ visual acuity was reviewed. In patients with primary vasculitis, visual improvement was noted in 3 months post-treatment, with 10.64% of eyes in the range of 6/6 – 6/18. 

Diagnosing and treating retinal vasculitis can be difficult for ophthalmologists, as untreated cases can lead to severe vision loss. Ophthalmologists can also play a crucial role in detecting underlying systemic diseases, enabling prompt treatment. Tailored treatment plans are necessary for each patient based on their specific findings, requiring a multidisciplinary approach when systemic involvement is present. Although systemic steroids effectively manage active disease and are easily administered, concerns over potential adverse systemic complications arise. In our two-year study, we established a three-month criterion for evaluating treatment outcomes. Longer-term studies are needed to address these limitations, and ongoing research aims to further refine our approach to retinal vasculitis.

Conflict of interest:

There is no conflict of interest among the present study authors.

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