Takayasu's arteritis presenting as bilateral cataracts: report of three cases

L. F. Flores-Suárez, J. A. Simón, P. A. Reyes1, M. E. Soto1, C. Castañón2 and P. Navarro3

Departments of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán (INCMNSZ), Departments of
1 Immunology and
2 Ophthalmology, Instituto Nacional de Cardiología Ignacio Chávez and
3 Asociación para Evitar la Ceguera en México, México City, México

SIR, Cataracts have been seldom described as initial manifestation of Takayasu's arteritis (TA). We describe three cases which presented due to rapidly evolving cataracts leading to blindness.

Case 1 was a 25-year-old woman who developed diminished visual acuity over a 5-month period. On examination she could only count fingers at 15 cm with both eyes, had unreactive dilated pupils with bilateral opacification of lenses (Fig. 1AGo) and bilateral flare with no rubeosis of the angle or iris. Right-eye intraocular pressure (REIOP) was 16 mmHg, left-eye intraocular pressure (LEIOP) 17 mmHg. After cataract excision and lens implants in both eyes her visual acuity improved to finger-counting at 2–3 m. Funduscopy showed marked venous stasis and de novo vessel formation. On fluoroangiography (FAG), peripheral and medial retinal ischaemia, bilateral papillary hyperfluorescence and prolonged arteriovenous filling time were seen; arteriovenous anastomoses were documented. As murmurs were noticed on the neck, limbs, abdomen and inguinal areas, panarteriography followed. The findings were: occlusion of both subclavian and carotid, anterior descendant coronary, both renal, infrarenal abdominal aorta and right common iliac arteries (Numano V TA) [1]. Her disease course was complicated by cardiac insufficiency and she died in September 2001.



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FIG. 1. (A) Total mature intumescent cataract secondary to ischaemia (case 1). (B) Funduscopy performed after cataract excision shows vast hypoxic areas, neovascularization of the optic disc and arteriovenous shunts, the hallmark of Takayasu's retinopathy (case 3).

 
Case 2 was a 31-year-old woman who presented with sudden bilateral loss of vision of 3 months' duration. On examination she only perceived light, and had unreactive pupils, bilateral enophthalmos due to gross hypotony and leukocoria due to complete cataracts which precluded funduscopy; there was no rubeosis of the iris, but both irises were atrophic. REIOP was 12 mmHg and LEIOP was 10 mmHg. Absence of limb pulses was found. Angiography showed complete occlusion of the brachiocephalic trunk and the common left carotid and subclavian arteries (Numano I TA). She has been treated with prednisone and intravenous cyclophosphamide.

Case 3 was a 21-year-old woman who presented with an 8-month-history of reduced vision in the left eye. Visual acuity was hand movement, and unreactive dilated pupil and anterior chamber flare were documented. No angle or iris rubeosis were found, but the latter was atrophic; a total mature cataract was seen. REIOP was 13 mmHg and LEIOP 16 mmHg. After left-eye cataract excision, visual acuity did not improve. Right-eye visual acuity was 20/30. Funduscopy showed disc neovascularization with shunts and retinal ischaemia in the left eye (Fig. 1BGo), fluoroangiography showing retinal hypoperfusion, papillary hyperfluorescence and prolonged arteriovenous filling. Right-eye findings were normal. She had no neck or arm pulses. Panarteriography showed total occlusion of the left common carotid and subclavian arteries and 80% occlusion of the brachiocephalic arterial trunk (Numano I TA). In September 2002 successful aorto-right carotid and subclavian bypass surgery was performed.

Cataracts have been described in association with TA, mostly after many years of evolution, rarely antedating other manifestations. They have been attributed to the patients' age and chronic treatment with steroids [2]. In one series in which cataracts were described in 15 eyes from 65 patients, the mean age of the patients was 50.2 years, with a mean time of disease evolution of 16.8 years [2]. In unpublished observations, we have seen cataracts in seven out of 83 patients (8.4%), mostly related to the above-stated factors.

This series is unique as the patients sought attention because of visual impairment from rapidly progressive cataracts, which were bilateral in two of the patients. All patients were young, had not received any treatment and, on average, they had had symptoms for 5 months. Previously, only one TA case has been described in which the patient presented with monocular blurred vision [3].

Aside from the cataracts, two of our patients had severe Takayasu's retinitis (TR), which leads us to think that the ischaemia seen in TR developed before the cataracts. It is well known that the production of vascular endothelial growth factor (VEGF) is stimulated in experimentally induced or retinal–ischaemic diseases, such as diabetic retinopathy [4]. In the report by Harada et al. [3], elevated serum levels of VEGF were found, although its contribution to cataract is unclear. We were not able to measure this cytokine. Chronic retinal ischaemia could have led to hypersecretion of VEGF, which in turn led to the neovascularization found in our patients' retinas. The circulatory alterations could also diminish the nutrition of the lens, leading to metabolic changes and possibly neuroreceptor derangement, favouring cataract formation. These mechanisms might also operate in other diseases in which retinal ischaemia may be present, irrespective of the aetiology. We did not observe iris rubeosis, but atrophy and flare were present in two patients each, indicating anterior chronic ischaemic changes which could contribute to cataracts.

We conclude that, although cataract is rarely reported in TA, its appearance can be abrupt and proceed to visual loss. It seems that in cases that are not related to age or treatment, the presence of cataracts confers an adverse visual prognosis, as severe TR seems to be invariably present [5, 6]. Therefore, we emphasize the importance of considering TA in the differential diagnosis of young patients presenting with severe cataracts, especially when bilateral. Subsequent adequate diagnostic workup can establish TA as the cause of cataracts and evaluate other sites of involvement amenable to proper treatment and follow-up.

Notes

Correspondence to: L. F. Flores-Suárez, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Sección XVI, Tlalpan, México, D.F. C.P. 14000, México. E-mail: felipe98{at}prodigy.net.mx Back

References

  1. Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol 1996;54(Suppl. 1):S155–63.[CrossRef][Medline]
  2. Kiyosawa M, Baba T. Ophthalmological findings in patients with Takayasu disease. Int J Cardiol 1998;(Suppl. 1):S141–7.[CrossRef]
  3. Harada M, Yoshida H, Mitusyama K et al. Aortitis syndrome (Takayasu's arteritis) with cataract and elevated serum level of vascular endothelial growth factor. Scand J Rheumatol 1998;27:78–9.[CrossRef][ISI][Medline]
  4. Aiello LP, Avery RL, Arrigg PG et al. Vascular endothelial growth factor in ocular fluid of patients with diabetic retinopathy and other retinal disorders. N Engl J Med 1994;331:1480–7.[Abstract/Free Full Text]
  5. Sagar S, Kar S, Gupta A, Sharma BK. Ocular changes in Takayasu's arteritis in India. Jpn J Ophthalmol 1994;38:97–102.[ISI][Medline]
  6. Bapat VN, Tendolkar AG. Turning a blind eye. Lancet 1997;349:1740.[CrossRef][ISI][Medline]
Accepted 11 December 2002





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