Acquired cytomegalovirus (CMV) retinitis is almost invariably a disease ofthe immunocompromised, now being seen most commonly in patients with AIDS or ...
Journal of the Royal Society of Medicine Volume 86 May 1993
Retinal neovascularization secondary to cytomegalovirus retinitis in Wegener's granulomatosis
P Riordan-Eva FRCS FCOphth' C E Williams FCOphth' A J Wing FRCP2 E M Graham MRCP MCOphth' M D Sanders FRCP FRCS1 'Medical Eye Unit and 2Renal Unit, St Thomas' Hospital, London Keywords: cytomegalovirus retinitis; retinal neovascularization
Acquired cytomegalovirus (CMV) retinitis is almost invariably a disease of the immunocompromised, now being seen most commonly in patients with AIDS or organ transplant recipients. It is a recognized complication of immunosuppression for systemic inflammatory disorders"4. CMV retinitis in a patient with Wegener's granulomatosis resulted in the previously unreported complication of aggressive proliferative retinopathy leading to vitreous haemorrhages and traction retinal detachment. Retinal ischaemia due to retinal vascular occlusions was the cause of the neovascularization. Case report In 1983 a 63-year-old male Caucasian developed chronic nasal discharge with recurrent epistaxis, lethargy, weight loss, microscopic haematuria and proteinuria. Nasal mucosal biopsy showed granulomas and giant cells and renal biopsy showed focal segmental proliferative glomerulonephritis with occasional crescents. He was diagnosed as having Wegener's granulomatosis, and treated with varying combinations of prednisolone, azathioprine, and cyclophosphamide. (Cytoplasmic pattern anti-neutrophil cytoplasmic antibodies (C-ANCA) were subsequently detected at a titre of 1: 8 while the disease was in remission.) In March 1988 the patient developed bilateral inflammatory retinitis. A clinical diagnosis of cytomegalovirus infection was substantiated by a rise in serum CMV complement fixing IgG titre initially from 1 :32 to 1: 128 over 10 days and subsequently to 1 : 16 300, and isolation of CMV from throat swab and urine specimens. Human immunodeficiency virus antibody test was negative. Reduction of the immunosuppressant therapy and a 2 week course of intravenous
Figure 1. Left eye, May 1988. Fluorescein angiogram: ischaemic macula (arrow) without evidence of active retinitis
Correspondence to: Mr P Riordan-Eva, FRcs FCOphth, Department of Ophthalmology, The National Hospital for Neurology and Neurosurgery, Queen Square, London WClN 3BG
Figure 2. Left eye, August 1988. Fluorescein angiogramr widespread vascular closure at the macula (large closed arrow) and peripherally (small closed arrows), with disc (large open arrow) and peripheral new vessels (small open arrows), in the absence of active retinitis
ganciclovir 5 mg/kg twice daily successfully controlled the retinitis. In May 1988 the visual acuity of the left eye dropped to 4/60 due to macular ischaemia, as demonstrated by fluorescein angiography (Figure 1), without any evidence of active retinitis at the macula although the retinitis had reactivated in the peripheral retina of both eyes. Ganciclovir was restarted at a dose of 5 mg/kg twice a day, reducing after 2 weeks to a maintenance dose of 5 mg/kg once a day. In August 1988 disc and peripheral new vessels were noted in the left eye. These had developed despite control of the peripheral retinitis. Fluorescein angiography showed widespread vascular closure (Figure 2). Vitreous haemorrhages in the left eye in September and October 1988 were followed by the development of extensive traction retinal detachment. Vision was reduced to hand movements. Acuity in the right eye was 6/12. Remission of the Wegener's granulomatosis allowed discontinuation of the immunosuppressant therapy in September 1988 and the ganciclovir in November 1988. Discussion CMV retinitis is a recognized complication of immunosuppressive therapy, and may cause profound bilateral visual loss. Treatment involves reduction of levels of immunosuppression where possible and administration of ganciclovir, which may need to be continued as maintenance therapy once retinitis has become quiescent and until immunocompetence is restored. Retinal neovascularization develops in response to retinal ischaemia or intraocular inflammation. Although this patient had inflammatory eye disease, the clinical course indicates that the stimulus to neovascularization was retinal ischaemia secondary to retinal vascular occlusions. Retinal vascular changes are very rarely seen in Wegener's granulomatosis5, except in its necrotizing sarcoidal type6, which was not present in this patient. Also there was no evidence of diabetes mellitus, systemic hypertension, or carotid artery disease to explain the retinal vascular disease. CMV retinitis is frequently accompanied by retinal vascular changes, and infection of endothelial cells by CMV has been demonstrated histologically in CMV retinitis in non-AIDS patients7'8. It is suggested that the retinal vascular changes in this patient were due to the CMV infection and they were responsible for the widespread retinal ischaemia leading to gross retinal neovascularization, vitreous haemorrhages, and traction retinal detachment; a course of events that has not previously been described in CMV retinitis. References 1 Nicholson DH. Cytomegalovirus infection of the retina. Int Ophthalmol Clin 1975;15:151-62 2 Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Ophthalmology
1983;90:279-90
301
302
Journal of the Royal Society of Medicine Volume 86 May 1993
3 Winkler A, Finan MJ, Pressly T, Roberts R. Cytomegalovirus retinitis in rheumatic disease: A case report. Arthritis Rheum 1987;30:106-8 4 Scott WJ, Giangiacomo J, Hodges KE. Accelerated cytomegalovirus retinitis secondary to immunosuppressive therapy. Arch Ophthalmol 1986;104:1117-24 5 Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocular manifestations of Wegener's granulomatosis. Am J Med 1977; 63:131-41 6 Spalton DJ, Graham EM, Page NGR, Sanders MD. Ocular changes in limited forms of Wegener's granulomatosis. Br J Ophthalmol 1981;65:553-63
7 de Venecia G, Zu Rhein GM, Pratt MV, Kisken W. Cytomegalic inclusion retinitis in an adult. Arch Ophthalmol 1971;86:44-57 8 Aaberg TM, Cesarz TJ, Rytel MW. Correlation of virology and clinical course of cytomegalovirus retinitis. Am J Ophthalmol 1972;74:407-15
(Accepted 15 January 1992)
