Abstract
A man in his 40s with blurry vision in both eyes presented with headaches, arthritis, anorexia, and weight loss. Visual acuity was counting fingers OD and hand motions OS. Examination revealed a flat nasal bridge, anterior chamber cells, and vitreous cells (in addition to the posterior segment findings described in the Figure legend). Workup revealed antineutrophil cytoplasmic antibody (proteinase 3 antineutrophil cytoplasmic antibody) positivity, consistent with granulomatosis with polyangiitis. Treatment with systemic steroids was initiated and transitioned to methotrexate, cyclosporine, and infliximab. The systemic symptoms and ophthalmic findings improved. Figure. View LargeDownload A, Ultra-widefield photography demonstrates disc edema, dilated and tortuous retinal vasculature, extensive subretinal turbid fluid, serous retinal detachment, and choroidal detachments. B, Treatment with immunomodulatory therapy resulted in resolution of the exudative process, leaving a wide net of subretinal fibrosis. Granulomatosis with polyangiitis, formerly Wegener granulomatosis, is a systemic vasculitis that affects small and medium vessels. The most common ophthalmic manifestations are orbital disease and scleritis.1-4 The images of this case illustrate a severe manifestation of the disease, which was treated with aggressive imunomodulatory therapy.5 Although this potentially fatal disease is rare, recognizing the findings might not only save the eye but might also be life-saving.5 Back to top Article Information Corresponding Author: Lisa J. Faia, MD, Associated Retinal Consultants, William Beaumont Hospital, 3535 W Thirteen Mile Rd, Ste 344, Royal Oak, MI 48073 (lfaia@arcpc.net). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. References 1. Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978;89(5 Pt 1):660-676.PubMedGoogle ScholarCrossref 2. Straatsma BR. Ocular manifestations of Wegener’s granulomatosis. Am J Ophthalmol. 1957;44(6):789-799.PubMedGoogle ScholarCrossref 3. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener’s granulomatosis. Ophthalmology. 1983;90(3):279-290.PubMedGoogle ScholarCrossref 4. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology. 1995;102(4):687-692.PubMedGoogle ScholarCrossref 5. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98(1):76-85.PubMedGoogle ScholarCrossref
Journal
JAMA Ophthalmology
– American Medical Association
Published: Jul 1, 2016
Keywords: wegener granulomatosis,weight reduction,diagnostic imaging,headache,blurry vision,arthritis,loss of appetite,anorexia nervosa,eye
