Retinal artery occlusion in Wegener's granulomatosis

P. Lamprecht, C. Lerin-Lozano, E. Reinhold-Keller, B. Nölle1 and W. L. Gross

Poliklinik für Rheumatologie, Universitätsklinikum Lübeck, Medizinische Universität Lübeck and Rheumaklinik Bad Bramstedt, Oskar-Alexander-Strasse 26, 24576 Bad Bramstedt and
1 Universitäts-Augenklinik, Klinik für Ophthalmologie, Christian-Albrechts-Universität Kiel, Hegewischstrasse 2, 24105 Kiel, Germany

SIR, We read with interest the article of O'Gradaigh et al. [1] reporting the occurrence of blindness in Wegener's granulomatosis due to retinal artery occlusion. We should like to add another case of retinal artery occlusion in a patient with Wegener's granulomatosis. In contrast to the patients reported by O'Gradaigh et al. [1], our patient had a favourable outcome with regard to his visual impairment.

A 57-yr-old patient had suffered a cough and waxing and waning pulmonary infiltrates on chest X-rays for 1 yr, after which generalized Wegener's granulomatosis with pulmonary–renal syndrome developed within 3 weeks. He was admitted with arthralgia, myalgia, malaise, fever, temporal headache, nasal obstruction, bloody nasal discharge, haemoptysis, new deafness and sensory peripheral polyneuropathy. Pulmonary infiltrates and nodules were seen on a chest X-ray and computed tomography scans, and bronchoalveolar lavage demonstrated alveolar haemorrhage. He had mild proteinuria, dysmorphic microhaematuria with red blood cell casts, oliguria, new hypertension and a creatinine concentration of 386 µmol/l. The erythrocyte sedimentation rate was elevated and mild leukocytosis (12 456/µl) was present. A cytoplasmic pattern antineutrophil cytoplasmic antibody (C-ANCA) (1:64) was detected by indirect immunofluorescence and antibodies to proteinase 3 (PR3-ANCA >128 U/ml) by enzyme-linked immunosorbent assay. Transbronchial and nasal biopsy specimens showed granuloma formation and necrotizing pauci-immune vasculitis. High-dose intravenous corticosteroid therapy (1 g prednisolone i.v. daily for 3 days) and oral cyclophosphamide (2 mg/kg p.o. per day) was started (Fauci's regimen). The prednisolone dose was then tapered (2 mg/kg p.o. per day). On the fifth day of therapy the patient complained of blurred vision in the left eye, which at first could not be verified by measuring visual acuity. However, 6 days later there was sudden visual loss (Table 1Go). Fundoscopy revealed an occlusion of the central retinal artery. Cranial MRI scans demonstrated maxillary sinusitis but did not show any inflammatory tissue or granuloma within the orbits. High-dose intravenous prednisolone therapy and intensified oral cyclophosphamide therapy (4 mg/kg, i.e. Fauci's regimen) was employed, and vision slowly improved during the following 6 months. The prednisolone and cyclophosphamide doses were tapered in accordance with the improving vision; there was no relapse of the visual loss. However, a peripheral visual field defect remained. When remission was achieved after 1 yr, the patient was switched to methotrexate and has remained in remission during follow-up for 11 months.


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TABLE 1. Visual acuity during oral cyclophosphamide and corticosteroid therapy of retinal artery occlusion due to retinal vasculitis in a patient with Wegener's granulomatosis

 
Ocular involvement has been reported to be present in 52% of patients with Wegener's granulomatosis during the entire course of illness [2, 3]. Episcleritis, scleritis, corneal ulceration, uveitis, retinal vasculitis, optic neuropathy and spreading of granulomatous masses from the sinus per continuitatem into the orbita with proptosis and secondary optic nerve ischaemia, dacryoadenitis, and obstruction of the nasolachrymal duct are features of ocular involvement in Wegener's granulomatosis [3, 4]. We found ocular involvement, i.e. episcleritis and/or orbita granuloma formation, in 94 of 155 (61%) patients with Wegener's granulomatosis during the course of their illness. However, there was evidence of retinal artery occlusion due to retinal vasculitis in only one patient. The notion that a peripheral visual field defect remained after effective treatment in this patient may further indicate the presence of an additional partial optic nerve atrophy.

The case reported here adds to the few cases of retinal artery occlusion in Wegener's granulomatosis reported so far [1]. Our patient received high-dose intravenous prednisolone pulse therapy and intensified oral cyclophosphamide therapy after visual acuity had been impaired during tapering of the initial steroid dose. Vision was regained by intensified immunosuppressive therapy, and another relapse was avoided.

The effect of cyclophosphamide does not become apparent until 7–10 days after the first administration. Tapering of the concomitant prednisolone dose during this time may result in relapse or progression of insufficiently suppressed disease manifestations during this critical period of therapy. In patients with a severe course of Wegener's granulomatosis, cyclophosphamide and corticosteroids may have to be administered at doses exceeding the standard treatment (i.e. using the intensified protocol of Fauci's regimen) until clinical improvement is achieved or limitations such as leukopenia occur [5]. In an earlier study, pulse cyclophosphamide therapy was found to be effective only in patients with moderate disease activity; severe and rapidly progressive forms of Wegener's granulomatosis responded better to daily oral cyclophosphamide [6].

Our patient had also complained of temporal headache. Temporal headache, sudden visual loss and (partial) optic nerve atrophy are clinical features of cranial vasculitis with secondary temporal arteritis and ophthalmic arteritis. Secondary temporal arteritis has been reported in a few patients with ANCA-associated vasculitides [7, 8] and other vasculitides [9]. Headache may be attributed to sinusitis, otitis or orbita granuloma in Wegener's granulomatosis, but secondary arteritis temporalis and ophthalmic arteritis, threatening vision, should also be considered, as demonstrated in our case. This situation may necessitate immediate and vigorous therapy in order to avoid permanent visual loss.

Notes

Correspondence to: P. Lamprecht, Poliklinik für Rheumatologie, Universitätsklinikum Lübeck, Medizinische Universität Lübeck and Rheumaklinik Bad Bramstedt, Oskar-Alexander-Strasse 26, 24576 Bad Bramstedt, Germany. Back

References

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