Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s granulomatosis

Abstract

BackgroundGranulomatosis 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 presentationA 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.ConclusionVasculitis-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.

Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

“…Central retinal vein occlusion has also been reported in younger people with GPA, although the mechanism remains unclear (48). Significant angiopathy and retinal hemorrhages could be the presenting sign of GPA (49). Although many chorioretinal manifestations are of vasculitic origin, with retro-orbital involvement lost vision (19).…”

Ocular Findings in a Patient with Wegener Granulomatosis: Case Report

“…Göz tutulumlarını saptamak diğer organlardaki aktif hastalığın habercisi olabileceğinden önem taşımaktadır. 4,7,8 Bunun yanında konjonktival biyopsinin WG tanısı koymada yardımcı olabileceği bildirilmektedir. 9 WG'de koroidal tutulum üveit, koroidal foldlar, retina pigment epitel değişiklikleri, koroidal arteriyel tıkanıklık, koryokapillarit ya da koroidal tümör olarak ortaya çıkabilmektedir.…”

“…Ayrıca, sklero-koroidal granülomlara ya da arka sklerite bağlı koroidal foldlar, koroidal effüzyon ve eksüdatif retina dekolmanı görülebilmektedir. 4,7 Sağ gözde skleral incelme, ön kamarada 3+ hücre ve koroiditin bulunması ve retina tutulumuna bağlı görülen arterit, retinal ven dal tıkanıklığı ile atılmış pamuk manzarası görünümü gibi bulguların olmaması, olgumuzda üveal tutulumun ön planda olduğunu dü-şündürmektedir. 3,4,11 Tedavi de hem indüksiyon fazı hem de idame fazında siklofosfamid, metotreksat ya da azatiopü-rin gibi immünsupresif ilaçlar kortikosteroidler ile kombine edilerek kullanılır.…”

Papillophlebitis with Uveitis in MPO ANCA Associated Pregnancy

“…Papillophlebitis has also been documented in uncomplicated pregnancy as a selflimiting condition [5]. Vasculitis-like retinal changes with hemorrhage can occur in systemic conditions like Wegener's granulomatosis /granulomatosis with polyangiitis responding well to immunotherapy (WG/GPA) [6]. The overall incidence of WG/GPA is estimated to be 4-8.8 cases / million but this varies depending on location.…”