Vasculitis-like hemorrhagic retinal angiopathy in Wegener's granulomatosis.

Abstract

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.

• Authors: Juliane Matlach, Florentina J Freiberg, Ottar Gadeholt, Winfried Göbel
• Journal: BMC research notes (BMC Res Notes) Vol. 6 Pg. 364 (Sep 10 2013) ISSN: 1756-0500 [Electronic] England
• PMID: 24021028 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Antibodies, Antineutrophil Cytoplasmic
• Immunosuppressive Agents
• Cyclophosphamide
• C-Reactive Protein

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Antibodies, Antineutrophil Cytoplasmic (blood)
• Blood Sedimentation
• C-Reactive Protein (metabolism)
• Cyclophosphamide (therapeutic use)
• Granulomatosis with Polyangiitis (complications, drug therapy, immunology, pathology)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Neutrophils (immunology, pathology)
• Retinal Hemorrhage (complications, drug therapy, immunology, pathology)
• Retinal Vessels (pathology)
• Vasculitis (complications, drug therapy, immunology, pathology)