Idiopathic unilateral ocular vasculitis in a young adult: a case report on clinical course and steroid response

Abstract

Introduction and importance:

Ocular vasculitis is an inflammatory condition affecting intraocular blood vessels, commonly associated with systemic diseases like Behçet’s disease, sarcoidosis, and tuberculosis. However, idiopathic isolated ocular vasculitis is rare, particularly in young adults, and presents diagnostic difficulties. Timely recognition is vital to prevent vision loss.

Case presentation:

We present a 25-year-old male with unilateral, painless, progressive visual blurring and floaters in the left eye. Examination showed anterior uveitis and retinal vasculitis, including perivasculitis, venous sheathing, and subretinal exudates. Extensive systemic evaluation, including autoimmune and infectious screening, was negative. A diagnosis of idiopathic ocular vasculitis was made. The patient received a single posterior sub-Tenon injection of triamcinolone acetonide along with topical anti-inflammatory agents. Significant clinical improvement was observed, with visual acuity improving from 6/18 to 6/9 and resolution of inflammatory signs.

Clinical discussion:

This case highlights a rare presentation of idiopathic ocular vasculitis involving both anterior and posterior segments, without systemic association. Idiopathic cases are typically diagnosed by exclusion. While systemic corticosteroids are commonly used for vasculitis, localized steroid therapy can be safer and equally effective in isolated ocular cases. The patient’s rapid response supports the role of targeted local therapy.

Conclusion:

Idiopathic ocular vasculitis, though rare, should be considered in patients with intraocular inflammation and no systemic findings. Exclusion of secondary causes and timely localized steroid treatment can preserve vision and prevent complications. Individualized management remains key in such rare presentations. Autoimmune and infectious causes including tuberculosis, syphilis, toxoplasmosis, and connective tissue disorders were excluded through thorough laboratory investigations.

The patient was followed up for 6 months, demonstrating complete resolution and no recurrence, strengthening the role of local corticosteroid therapy in idiopathic cases.

Introduction

Ocular vasculitis involves intraocular vessels in the process of becoming inflamed and may manifest as anterior, intermediate, posterior, and also pan-uveitis, often extending itself to the retinal, choroidal, and iris vasculature^[1]. Behçet’s disease, sarcoidosis, as well as tuberculosis exist as systemic or infectious conditions. Retinal vasculitis is often secondary to these conditions, also to vasculitides such as systemic lupus erythematosus^[2]. Rare idiopathic cases account for just about 1% of patients, while around 8% show syndromic associations, according to a meta-analysis recently done^[3].

HIGHLIGHTS

• Rare case of unilateral idiopathic ocular vasculitis in a healthy young male.

• Inflammation affected both anterior uveitis and retinal vasculitis.

• Posterior sub-Tenon triamcinolone led to rapid symptom and vision improvement.

• Systemic and infectious evaluations were negative, confirming idiopathy.

• Highlights early diagnosis, systemic exclusion, and local steroid treatment.

Clinically, retinal vasculitis of an idiopathic nature often affects some young adults and typically presents with a set of symptoms which are gradual vision decline, floaters, and discomfort of the ocular area, in conjunction with some key fundus findings including retinal perivasculitis, venous tortuosity, vascular sheathing, and subretinal exudation^[4]. These findings are confirmed and monitored by fluorescein angiography (FA) and optical coherence tomography (OCT).

Corticosteroid therapy remains as the foundation of treatment in idiopathic cases though management has no well-defined guidelines. Specifically, posterior sub-Tenon injection of triamcinolone acetonide (PSTK) delivers high-dose steroids in such a targeted way. It reduces systemic exposure when directed toward posterior segment inflammation^[5]. Moreover, PSTK has shown efficacy in managing uveitis-related macular edema and posterior segment inflammation, with notable improvements in visual acuity and retinal anatomy. A prospective study from the United Kingdom demonstrated significant reduction in macular edema and visual improvement in patients with non-infectious posterior uveitis following PSTK treatment^[6].

In this report, we present a case of a 25-year-old man who presented with unilateral idiopathic ocular vasculitis, exhibiting blurred vision, pain, and inflammatory signs including anterior chamber cells, subretinal exudates, perivasculitis, and vessel sheathing. After comprehensive systemic workup excluded underlying disease, the patient received a single posterior sub-Tenon Kenacort injection, alongside topical anti-inflammatory agents. The intervention resulted in substantial improvement in visual acuity (from 6/18 to 6/9) and rapid resolution of inflammation, reinforcing the value of early localized therapy in idiopathic presentations. This case report has been reported in line with the CARE 2023 Guidelines^[7]

A systematic literature search was conducted across databases PubMed and Google Scholar (2010–2024) using the keywords “idiopathic ocular vasculitis,” “retinal vasculitis,” and “posterior sub-Tenon triamcinolone” to find similar cases and treatment findings.

Case presentation

A 25-year-old male, presented to the outpatient clinic with a 1-week history of gradually progressive, painless blurring of vision in the left eye, with occasional floaters and no associated photophobia or redness. He denied systemic symptoms including fever, joint pain, rash, weight loss, or cough. There was no relevant past medical, ocular, surgical, or family history, and no prior trauma or features suggestive of systemic autoimmune disease.

On ocular examination, best-corrected visual acuity (BCVA) was 6/6 in the right eye and 6/18 in the left, further reducing to 6/60 on pinhole testing in the left eye. Intraocular pressure was 12 mm Hg bilaterally by Goldmann applanation tonometry. Slit-lamp examination of the left eye revealed fine keratic precipitates and +2 anterior chamber cells, indicating anterior uveitis. The right eye appeared normal. Fundus examination of the left eye showed mild vitritis, subretinal exudates near the macula, retinal perivasculitis with venous tortuosity, peripheral vascular sheathing, peripapillary vessel sheathing, and prominent periretinal vitritis with vitreous condensation obscuring the macular veins. These findings were further supported by optical coherence tomography (OCT), which revealed increased central retinal thickness, disruption of the foveal contour, and subretinal exudates in the left eye (Fig. 1).Given these findings, a systemic workup was initiated to explore potential infectious or inflammatory etiologies.

Figure 1.

Optical coherence tomography (OCT) confirmed increased central macular thickness, subretinal exudation, and vitreoretinal interface irregularities, correlating with observed clinical findings of active vasculitis.

Laboratory investigations were unremarkable. Complete blood count demonstrated hemoglobin levels of 17.7 g/dl, hematocrit 56.6%, RBC 6.44 × 10⁶/μl, WBC 10.2 × 10⁹/l, and platelets 351 × 10⁹/l. ESR was 5 mm/hr.

Venereal Disease Research Laboratory (VDRL) test for syphilis was 0.02 IU/ml (negative); Toxoplasma IgM and IgG levels were 0.12 and 0.23, respectively (non-reactive); Quantiferon-TB Gold assay for tuberculosis was 0.09 IU/ml (negative); and the serum angiotensin-converting enzyme (ACE) level for sarcoidosis was 37.6 U/l (within the normal range of 8–52 U/l). Autoimmune and connective tissue disease screening was also negative, with rheumatoid factor (RF) <3.5 IU/ml (normal <14 IU/ml), antinuclear antibody (ANA) negative, and anti–double-stranded DNA (anti-dsDNA) negative. These results altogether excluded infectious and autoimmune causes, supporting the diagnosis of idiopathic ocular vasculitis.

The patient was followed every month for 6 months. Intraocular pressure (IOP) remained between 10–14 mm Hg, and best-corrected visual acuity (VA) was maintained at 6/9. No recurrence or complications were seen. Contrast sensitivity testing was not performed, which is a limitation of this report.

Discussion

Idiopathic ocular vasculitis is a rare, inflammatory condition that presents significant diagnostic and therapeutic challenges. It typically affects young adults and lacks identifiable systemic associations^[8]. Our patient, a 25-year-old male, presented with unilateral, painless, and progressively worsening blurring of vision, accompanied by floaters and vitritis. Blurred vision is the most commonly reported symptom in retinal vasculitis (RV), although clinical manifestations may vary depending on the site and extent of vascular involvement and the presence of complications such as vitreous hemorrhage or vitritis^[9].

Retinal vasculitis can affect arteries, veins, or capillaries, or manifest as diffuse vascular inflammation^[10]. Venous involvement, as observed in this case, is commonly reported and is often indicated by findings such as perivasculitis, venous tortuosity, and vascular sheathing. Peripheral vessel sheathing, which appeared as white cuff-like exudates surrounding the vessels on fundus examination, reflects perivascular infiltration by inflammatory cells and is considered a hallmark feature of RV^[7] (Fig. 2). Involvement of macular vessels is often responsible for central visual loss, whereas inflammation limited to peripheral retinal vessels may lead to peripheral leakage and subretinal exudates without significantly affecting vision^[10].

Figure 2.

Widefield fundus photograph of the left eye showing venous tortuosity, vascular sheathing, and subretinal exudates characteristic of retinal vasculitis.

The underlying pathophysiology of idiopathic ocular vasculitis remains largely unclear. Early observations of vascular sheathing and perivascular cuffing led researchers to propose a type III hypersensitivity mechanism, where immune complex deposition within retinal vessels initiates an inflammatory response^[11]. However, this hypothesis has not been consistently validated through human or animal studies^[12]. Instead, evidence suggests that retinal vasculitis involves a more complex interplay of immune pathways, incorporating both humoral and cell-mediated responses. Notably, CD4⁺ T cells have been found infiltrating the retinal tissue and surrounding the affected vessels, pointing toward a significant role of cell-mediated immunity in disease progression^[9]. In addition, genetic factors may also contribute to disease susceptibility, as some studies have reported a potential hereditary predisposition to developing retinal vasculitis^[13].

In our case, the disease presented unilaterally with predominant venous involvement. While venous-predominant RV is frequently described in literature, the unilateral nature of the condition is relatively uncommon. Isolated retinal vasculitis (IRV), in particular, tends to exhibit bilateral involvement. This is supported by Ermakova and Balishanskaia, who reported bilateral disease in 88.4% of patients with IRV in their cohort of 43 cases^[14,15]. Moreover, the presence of anterior chamber cells, in conjunction with retinal perivasculitis and vitritis, indicates active intraocular inflammation involving both anterior and posterior segments of the eye. This pattern of inflammation suggests that the disease process is not restricted to the retinal vessels alone but reflects a broader uveitic involvement. Such observations are consistent with previously reported cases in the literature, where anterior chamber inflammation often accompanies retinal vasculitis, reinforcing the concept of it being part of a more extensive inflammatory ocular syndrome rather than an isolated retinal condition^[14]. Notably, our patient also exhibited keratic precipitates (KPs), a finding that is typically uncommon in cases of purely idiopathic retinal vasculitis^[9]. Their presence further reinforces the involvement of the anterior segment in this case.

Retinal vasculitis may occur as an isolated ocular condition or as part of a systemic disease. Non-infectious systemic associations include autoimmune and inflammatory disorders such as sarcoidosis, Behçet’s disease, systemic lupus erythematosus (SLE), rheumatoid arthritis, multiple sclerosis, and seronegative arthritides. Infectious etiologies comprise tuberculosis, syphilis, Lyme disease, brucellosis, toxoplasmosis, and cat-scratch disease^[16]. Given this broad differential, a comprehensive diagnostic workup is essential to exclude underlying causes. In our case, detailed laboratory testing – including screening for syphilis, tuberculosis, and toxoplasmosis, as well as serum angiotensin-converting enzyme (ACE) levels – and imaging such as chest X-ray were all unremarkable. The absence of systemic symptoms such as fever, joint pain, rash, or weight loss further reduced the likelihood of an underlying autoimmune or infectious etiology. These negative findings, in the context of an otherwise healthy individual, supported the diagnosis of idiopathic ocular vasculitis. Systematic autoimmune and infectious screening consisting of rheumatoid factor, ANA, anti-dsDNA, Quantiferon-TB, VDRL, toxoplasma serology, and ACE were negative, validating the idiopathic nature of this case. This thorough assessment was necessary to exclude mimicking systemic causes.

It is crucial to recognize that other retinal conditions may mimic the clinical and angiographic features of RV. For instance, vascular leakage and perivascular changes may be observed in diabetic retinopathy, Coats’ disease, and retinal vascular occlusions. Vessel sheathing, although characteristic of vasculitis, may also appear in conditions such as branch or central retinal vein and artery occlusions due to other causes^[17,18]. Hence, accurate diagnosis often requires adjunctive imaging.

Fluorescein angiography (FA) plays a central role in the diagnosis and management of retinal vasculitis. It provides enhanced sensitivity compared to clinical examination, often revealing more extensive inflammation than initially suspected, and is vital in determining the extent of vascular leakage and ischemia^[14]. In our patient, FA findings confirmed the clinical impression of active vasculitis and guided targeted therapy.

The cornerstone of treatment for non-infectious retinal vasculitis is corticosteroid therapy, due to its potent and rapid anti-inflammatory effects^[19]. Our patient received a posterior sub-Tenon injection of triamcinolone acetonide, along with topical corticosteroids and NSAIDs. This therapeutic approach led to swift resolution of both anterior and posterior segment inflammation and significant improvement in visual acuity. This outcome is consistent with existing literature supporting the efficacy of local corticosteroid therapy in idiopathic ocular vasculitis. However, in cases with bilateral, recurrent, or severe inflammation – or where systemic disease is identified – systemic immunosuppressive agents such as methotrexate, azathioprine, or biologics may be required. These agents not only help achieve sustained disease control but also minimize long-term corticosteroid-related complications^[8]. Common side effects of posterior sub-Tenon triamcinolone include increased intraocular pressure, cataract progression, and rare scleral perforation; however, none was present in this patient.

Relapse rates of retinal vasculitis have been observed in up to 60% of cases within 1 year. Close follow-up is thus necessary to make sure sustained remission and early detection of any systemic disease evolution.

Strengths and limitations

This case reflects a rare idiopathic presentation of unilateral ocular vasculitis with both anterior and posterior involvement, confirmed by clinical and imaging findings. The use of localized posterior sub-Tenon triamcinolone therapy showed effective, indicating its role in non-systemic cases. However, the lack of long-term follow-up and advanced angiographic imaging restricts the generalizability and depth of vascular evaluation in this report.

Conclusion

This case highlights that idiopathic unilateral ocular vasculitis, though rare, can manifest with both anterior and posterior segment inflammation. Systematic exclusion of systemic and infectious causes is necessary before labeling a case idiopathic. Local corticosteroid therapy via posterior sub-Tenon injection proved safe and effective, leading to complete remission and preserved vision without recurrence over 6 months.

AI Use declaration

OpenAI’s ChatGPT (version GPT-4, July 2024) was used during the manuscript drafting process for language refinement, grammar correction, and formatting. All AI-assisted content was reviewed and approved by the authors to ensure scientific accuracy and ethical integrity. No patient data were entered into the AI tool.

Ethical approval

Ethical approval was not required for this case report, as all clinical investigations were conducted as part of routine patient care and in accordance with institutional guidelines.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying clinical images. A copy of the consent is available upon request from the Editor-in-Chief.

Sources of funding

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author contributions

S.N.U.S.: conceptualization, clinical case management, manuscript writing, final approval; Z.B.: literature review, case summary, data interpretation; A.H.: draft editing, referencing, and manuscript organization; A.K.: literature search and figure preparation; S.H.K.: data extraction, referencing support; F.H.: structure formatting, AI use declaration, and CARE compliance; S.H.U.: manuscript compilation, final approval and review; K.A.K.: manuscript review and critical revisions.

Conflicts of interest disclosure

The authors declare that there are no conflicts of interest related to the publication of this case report.

Research registration unique identifying number (UIN)

This is a case report and does not require research registration.

Guarantor

Syeda Noor Us Saba.

Provenance and peer review

Not commissioned; externally peer-reviewed.

Data availability statement

All data relevant to the case are included in the manuscript. Additional de-identified information may be provided upon reasonable request from the corresponding author.