Novel ultrawide field fundus fluorescein angiographic findings in a patient of Takayasu arteritis on immunosuppression.

A 35-year-old male, a case of Takayasu arteritis on treatment with oral prednisolone and methotrexate, revealed novel ultrawide field fluorescein angiographic features in bilateral eyes. The typical features of delayed arm-retina circulation time, lobulated areas of nonperfusion in choroid, cattle trucking of red blood cells in veins, and delayed arteriovenous transit time were nicely captured. Additionally, we noted peripheral perivascular hyperfluorescence and disc hyperfluorescence. We believe these findings could possibly be used as markers of disease activity. Lack of marked capillary nonperfusion areas and marked perivascular leakage probably signifies modulation of disease activity by the immunosuppressants.

Takayasu arteritis (TA), aka pulseless disease, is an idiopathic inflammatory vasculitis predominantly affecting young females with predilection for large- to medium-sized blood vessels.[1] Although the etiopathology is not well understood, it is believed to stem from an autoimmune granulomatous inflammation of the arteries resulting in attenuation and obliteration of the aorta and its branches.[2] The prevalence of ocular involvement in TA has been reported as ranging from 8.1% to 68% in different studies.[345] We here in report a case of TA (on treatment with immunosuppressants) and its vivid ocular vascular involvement using ultrawide field fundus fluorescein angiography (UWFFA).

Case Report

A 35-year-old male presented to our clinic with the chief complaint of decreased vision for the last 2 months. He was also complaining of weakness in upper extremities along with intermittent jaw claudication. He was a known case of TA on treatment. The patient was on oral methotrexate and prednisolone.

Initial general physical examination disclosed lack of arterial pulsation and unrecordable blood pressure in upper extremities. Blood pressure in the legs was about 110/58 mmHg.

The best corrected visual acuity on presentation was 20/40 in both eyes. Intraocular pressures were 10 mmHg in both eyes. Pupillary light reactions were normal. Slit lamp examination of anterior segment revealed presence of posterior subcapsular cataract bilaterally. Dilated fundus evaluation revealed few superficial hemorrhages in the periphery of left eye [Fig. 1b, arrow]. Mild hyperemia of optic disc along with venous dilation was also noted bilaterally [Fig. 1a and b]. There were no visible microaneurysms or exudates. UWFFA revealed increased arm to retina circulation time (22 s). Lobulated areas of choroidal nonfilling were seen in the arterial phase in bilateral eyes [Figs. 2b and 3b]. Disc hyperfluorescence was noted in early phase in bilateral eyes which increased with time [Serial Fundus fluorescein angiography (FFA) images Figs. 2a-c and 3a-c]. There was delayed arteriolar-venous transit time (5 s) bilaterally. In the late phase, the choroidal and retinal veins did show filling with mild diffuse perivascular hyperfluorescence, more in left eye. Some capillary nonperfusion (CNP) areas were also noted in the temporal retina in left eye. Cattle trucking of Red blood corpuscles (RBCs) in the veins was evident [Figs. 2d and 3d, arrow].

Figure 1

Ultrawide field image of right (a) and left eye (b) showing mild hyperemic disc with venous dilation. Left eye in addition revealing presence of flame shaped haemorrhages (arrow)

Figure 2

Serial UWFFA of right eye showing an increase arm to retina time of 22 s (a), lobulated areas of non-filling (arrow) in choroid in the arterial phase (b), increase in disc fluorescence with time (a-c) along with perivascular hyperfluorescence (red arrow) along vessels (c), and cattle trucking of RBCs in veins (arrow) can be well appreciated in the magnified view (d)

Figure 3

Serial UWFFA of left eye shows a similar increase arm to retina time of 22 s (a), lobulated areas of nonfilling in choroid in the arterial phase (b), increase in disc fluorescence with time (a-c) along with perivascular hyperfluorescence (red arrow) along vessels (c) with cattle trucking of RBCs in veins (arrow) in the magnified view (d)

Computed tomography angiogram revealed narrowing of right brachiocephalic trunk, common carotid arteries, complete occlusion of right subclavian artery just beyond right vertebral artery with focal stenosis of vertebral artery itself. Left vertebral artery was completely occluded from its origin. Estimation sedimentation rate was 32 mm/h. No ocular intervention was planned in view of absence of marked CNP in fundus or any signs of anterior segment ischemia. Patient was advised to follow-up regularly.

Discussion

We describe UFFA findings of a known case of TA on treatment with steroids and immunosuppressive agents. Some of the characteristic known fundus and fluorescein angiography findings which were also seen in our case were venous dilation, an increased arm to retina circulation time (22 s), and delayed arteriolar-venous transit time.[6] We could also clearly see slowing of flow in the veins with cattle trucking of RBCs in the veins [Figs. 2d and 3d arrow]. This finding has been previously described by Bajgai and Singh.[7]

UWFA also revealed well demarcated areas of lack of flow in the choroid starting from just beyond the arcades to the periphery in all quadrants [Figs. 2b and 3b]. These areas slowly filled with dye with time. This indicates slow choroidal flow as well. These are to be distinguished from wedge shaped areas of choroidal hypofluorescence seen in cases of blockage of posterior ciliary arteries (Amalricsign).[8] Mild diffuse perivascular hyperfluorescence along peripheral vessels was seen in late phases. Disc hyperfluorescence was also evident from early phase which increased with time; however, there was no obvious neovascularisation of the disc, also there was no evidence of disc edema or ischemic optic neuropathy.

The entire gamut of fundus and fluorescein angiographic findings seen in our case seems to be a result of the primary disease and its modulation as a result of therapy with immunosuppressive agents and steroids. These agents have probably kept the inflammation in the ocular vessels under control preventing development of occlusive vasculitis and CNP formation. The mild perivascular leak and the disc leak are still suggestive of some amount of persisting inflammation. Lack of marked capillary nonperfusion areas and marked perivascular leakage probably signifies suppression of disease activity by the immunosuppressants.

Conclusion

From one report it is difficult to say whether these UWFA findings can be used as a guide to adequate level of immunosuppression or not. However, a large series of wide field angiographic analysis of retinal vessels of such cases may reveal useful information on the correlation between therapy and perivascular leakage/development of capillary dropout areas. Thus, ultrawide field fluorescein angiography of cases of TA could potentially be used as an adjunctive marker of disease activity.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.