Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s granulomatosis

BMC Research Notes

Winfried Göbel¹  Ottar Gadeholt²  Florentina J Freiberg¹  Juliane Matlach¹ 

[1] Department of Ophthalmology, University of Wuerzburg, Wuerzburg, Germany;Department of Internal Medicine II, University of Wuerzburg, Wuerzburg, Germany

关键词: Cyclophosphamide;    Hemorrhages;    Retinal vasculitis;    Wegener’s granulomatosis;    Granulomatosis with polyangiitis;   

Others  :  1141686
DOI  :  10.1186/1756-0500-6-364

 received in 2012-12-27, accepted in 2013-08-29,  发布年份 2013

【 摘 要 】

Background

Granulomatosis with polyangiitis, also known as Wegener’s granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener’s granulomatosis).

Case presentation

A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener’s granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy.

Conclusion

Vasculitis-like retinal changes can occur in Wegener’s granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms.

【 授权许可】

   
2013 Matlach et al.; licensee BioMed Central Ltd.

【 图 表 】

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